Glycohemoglobin (HbAIc): a predictor of birth weight in infants of diabetic mothers

Hemoglobins AIa-c (fast Hb), minor variants of HbA, are elevated in patients with diabetes mellitus. Recent studies indicate a relationship of fast hemoglobins, especially HbAIc (glycosylated form), to chronic hyperglycemia. Since infant oversize has been attributed to maternal hyperglycemia and fetal hyperinsulinemia, the hemoglobin HbAIc fraction was compared to birth weight (actual and relative to gestational age) and to maternal glucose tolerance. Normal (13), probably normal (8), gestational diabetic (10), and insulin-dependent women (14) were studied in the third trimester; women with advanced diabetic vascular disease were excluded. When corrected for gestational age, relative birth weights correlated in a significant linear regression with HbAIc (n = 45, r = 0.57, P less than 0.001). Third trimester maternal glucose tolerance (Kt) of women, not insulin dependent, correlated in a signigicant manner with both HbAIc (P less than 0.05) and birth weight for gestational age (P less than 0.01).     

Screening for gestational diabetes mellitus

The term 'gestational diabetes mellitus' is unsatisfactory as it refers to a heterogeneous group of women, including those with minimal abnormality of carbohydrate metabolism and those with undiagnosed type II diabetes. However, perinatal morbidity is increased even in the group of women who have only impaired glucose tolerance; the mothers are at increased risk of subsequent development of diabetes, and there may also be long-term implications for the offspring. Current research is aiming to define the blood glucose levels at which risks increase so that clinical management can be appropriately directed. When available, the criteria required to justify population screening in pregnancy should be satisfied. The glucose challenge and fasting glucose tests are the leading contenders as appropriate screening tests to determine who should have the diagnostic glucose tolerance test. However, until this is reviewed, the widely used scheme of risk factors as a screening method should continue, as it detects at least 50% of women with gestational diabetes.     

Teenage pregnancy in type 1 diabetes mellitus

Carmody D, Doyle A, Firth RGR, Byrne MM, Daly S, Mc Auliffe F, Foley M, Coulter‐Smith S, Kinsley BT. Teenage pregnancy in type 1 diabetes mellitus Younger maternal age at delivery has been linked to adverse reproductive outcomes. Pregnancy complicated by type 1 diabetes mellitus (T1DM) is also associated with adverse pregnancy outcomes. Optimising diabetic glycaemic control prior to pregnancy is known to reduce the rate of congenital abnormalities and improve pregnancy outcomes. Teenage pregnancies are not usually planned and little data exist on teenage pregnancy complicated by T1DM. We sought to identify the glycemic control achieved in teenage pregnancy with T1DM and to clarify if there is an associated increase in adverse pregnancy outcomes compared to those seen in older women with T1DM. We compared outcomes in 18 teenagers (TG) with 582 older women with T1DM (CON) from 1995–2007. TG booked to the combined diabetes‐obstetrical service at a median gestational age of 11 weeks (range 6–22) compared to 7 weeks in CON (range 4–40, p < 0.02). Glycaemic was worse in TG compared to CON at 13, 26 and 35 weeks gestation, despite higher insulin doses. First trimester miscarriage rate did not differ between groups. Major congenital anomaly rate was 6.2% (1/16) compared to 3.2% in CON. This preliminary study has demonstrated that pregnant teenage women with T1DM book later to specialised care and have worse glycaemic control in pregnancy compared to older women with T1DM. This group also appear to be more insulin resistant than older women in early pregnancy. Our data would suggest that teenagers with type 1 diabetes mellitus may constitute a high‐risk group for adverse pregnancy outcomes.     

Growth and neurodevelopmental outcome of children born to mothers with pregestational and gestational diabetes

Diabetes during pregnancy may be associated with a high rate of congenital anomalies, disturbances of intrauterine growth and often post-natal neurobehavioral abnormalities in the offspring. The latter are associated with pregestational (PGD) as well as with gestational diabetes (GD). In this review we discuss the effects of maternal glucose intolerance on the long-term growth and development of the offspring. In well-controlled diabetes, birth weight is often within normal limits while in partially controlled diabetes newborns are often macrosomic. In PGD mothers with nephropathy, newborns tend to be born prematurely and small for gestational age (SGA). Offspring of diabetic mothers are often large and overweight in comparison to controls. Their long-term development is sometimes impaired. Delayed brain maturity is often observed in newborns of diabetic mothers compared to controls. The IQ scores of the children born to well controlled diabetic mothers are generally similar to that of control children. However, these children perform less well than controls in fine and gross motor functions. They also seem to have a higher rate of inattention and/or hyperactivity as observed by various tests and questionnaires. In our studies we found, in accordance with published literature, a negative correlation between the performance of the children born to mothers with PGD or GD on various neurodevelopmental and behavioral tests and the severity of maternal hyperglycemia as assessed by blood glycosylated hemoglobin levels and acetonuria. In conclusion: PGD or GD may adversely affect intrauterine and postnatal growth, attention span and motor functions of the offspring, but not their cognitive ability unless complicated by nephropathy or hypertension. These effects are negatively correlated with the degree of maternal glycemic control.     

Congenital malformations among infants whose mothers had gestational diabetes or preexisting diabetes

BACKGROUND: Diabetes type 1 is associated with an increased risk for infant congenital malformations. It is debated whether this is true also at gestational diabetes. AIMS: To study occurrence of congenital malformations in infants whose mothers had preexisting or gestational diabetes. STUDY DESIGN: A register study covering over 1.2 million Swedish births in 1987-1997 based on the Swedish health registries. SUBJECTS: We identified from the Medical Birth Registry 3864 infants born of women with preexisting diabetes and 8688 infants born of women with gestational diabetes. OUTCOMES MEASURES: Congenital malformations identified in the Medical Birth Registry, the Registry of Congenital Malformations, and the Hospital Discharge Registry. The rates of congenital malformations among these infants was compared with the population rates. RESULTS: At preexisting diabetes, the total malformation rate was 9.5% while the rate at gestational diabetes was similar to the population rate, 5.7%. At preexisting diabetes, certain conditions were more common than expected: orofacial clefts, cardiovascular defects, oesophageal/intestinal atresia, hypospadias, limb reduction defects, spine malformations, and polydactyly. For some of these conditions, an excess was found also for infants whose mothers had gestational diabetes. Infants with multiple malformations were in excess at preexisting diabetes but not at gestational diabetes but the specific type of malformations involved were similar in the two diabetes groups. CONCLUSIONS: It is suggested that in the group of gestational diabetes exists a subgroup with an increased risk for a diabetes embryopathy, perhaps due to preexisting but undetected diabetes type 2.     

Caudal regression syndrome - caudal agenesis

BACKGROUND: Neural tube defects are caused by complex genetic and environmental factors. The congenital anomaly most specific to pregnant women with diabetes mellitus is caudal regression syndrome. PATIENT: A 4-year-old boy with a history of mild delay in motor development presented with primary enuresis and encopresis. On physical examination, he had no sensory and motor deficits, but a short anal cleft. On questioning, the mother reported insulin-dependent diabetes mellitus during pregnancy. MRI of the spinal cord demonstrated a thoracic syringomyelia, a dysplastic conus medullaris, and an absence of coccyx and distal sacrum, called caudal regression syndrome or caudal agenesis. CONCLUSION: The caudal regression syndrome refers to sacral agenesis associated with spinal cord anomalies, e.g. syringomyelia. Sacral agenesis is marked by total absence of the coccyx and total or distal absence of the sacrum. An abnormal backside combined with a history of maternal diabetes mellitus in pregnancy is highly suggestive for the presence of caudal regression syndrome.     

妊娠期糖耐量异常对新生儿的影响

目的：妊娠期糖代谢异常可引起新生儿多种并发症。该文通过回顾性分析,探讨妊娠期不同程度糖代谢异常对新生儿的影响。方法：根据孕妇在孕24～28周时50 g葡萄糖筛查试验(GCT)及75 g葡萄糖耐量试验(OGTT)的结果,将其所分娩新生儿分为4组：妊娠期糖尿病组(GDM,182例)、妊娠期糖耐量减低(GIGT)1 h组(57例)、GIGT (2~3 h)组(156例)、GCT异常而OGTT正常(仅GCT异常组, 38例),并以糖代谢正常孕妇所分娩新生儿1 025例作为对照,对妊娠期不同程度糖代谢异常孕母的围产儿结局进行比较。结果：GIGT（1 h）组巨大儿、大于胎龄儿及小于胎龄儿发生率,以及低血糖、早产发生率明显高于对照组,与GDM组类似。GIGT（2~3 h）组和仅GCT异常组巨大儿、小于胎龄儿以及低血糖和早产的发生率明显低于GDM组,差异均有显著性意义,与对照组比较差异无显著性意义。GIGT(1 h)组新生儿低血糖和早产发生率明显高于GIGT (2~3 h) 组和仅GCT异常组, 差异有显著性意义。结论：母亲妊娠期糖代谢异常程度不同对新生儿的影响不同。孕母OGTT 试验1 h 单项血糖升高的糖耐量减低与妊娠糖尿病一样对新生儿具有危险性,可导致巨大儿、大于胎龄儿、小于胎龄儿及低血糖、早产的发生率增加。［中国当代儿科杂志,2009,11（3）：177-180］     

Obesity in pregnancy: implications for the mother and lifelong health of the child. A consensus statement

Obesity among pregnant women is becoming one of the most important women's health issues. Obesity is associated with increased risk of almost all pregnancy complications: gestational hypertension, preeclampsia, gestational diabetes mellitus, delivery of large-for-GA infants, and higher incidence of congenital defects all occur more frequently than in women with a normal BMI. Evidence shows that a child of an obese mother may suffer from exposure to a suboptimal in utero environment and that early life adversities may extend into adulthood. In September 2009, ILSI Europe convened a workshop with multidisciplinary expertise to review practices and science base of health and nutrition of obese pregnant women, with focus on the long-term health of the child. The consensus viewpoint of the workshop identified gaps and gave recommendations for future research on gestational weight gain, gestational diabetes, and research methodologies. The evidence available on short- and long-term health impact for mother and child currently favors actions directed at controlling prepregnancy weight and preventing obesity in women of reproductive ages. More randomized controlled trials are needed to evaluate the effects of nutritional and behavioral interventions in pregnancy outcomes. Moreover, suggestions that maternal obesity may transfer obesity risk to child through non-Mendelian (e.g. epigenetic) mechanisms require more long-term investigation.     

测定脐血脑钠肽对血糖异常孕妇的胎儿心功能评价

目的：探讨脐血脑钠肽（BNP）评价血糖异常孕妇胎儿心功能的可行性及高血糖对胎儿心功能的影响。方法：妊娠期血糖异常孕妇24例,包括妊娠期糖尿病18例和糖耐量受损6例,按分娩前血糖值的高低分为控制欠佳组（7例）和控制较好组（17例）,孕末期行胎儿超声心动图测量心脏相关指标;正常妊娠孕妇25例为对照组。于胎儿分娩时留脐血,测定BNP浓度。结果：血糖异常组胎儿脐血BNP值明显高于对照组,分别为（114.0±39.0）和（80.6±13.7） pg/mL（P<0.01）,其中血糖控制欠佳组高于控制较好组（142.1±44.1 pg/mL vs 102.4±31.2 pg/mL;P<0.01）。脐血BNP在妊娠期糖尿病和糖耐量受损两组间差异无显著性,与孕末期胎儿左室壁厚度呈正相关,与二尖瓣A峰、E/A比值分别呈正、负相关（r值分别为0.715,0.491和-0.507,P<0.05）。结论：妊娠期高血糖时脐血BNP水平明显升高,与孕妇血糖控制情况及胎儿心功能变化相关联,可反映胎儿心功能的潜在损害。良好血糖控制可能减轻这一影响。［中国当代儿科杂志,2009,11（10）：805-808］     

Disorders in Glucidic Metabolism and Congenital Heart Diseases: Detection and Prevention

The identification of gestational diabetes (GDM) through appropriate screening and its subsequent treatment have not been demonstrated to limit neonatal malformations to date. This study aimed to detect congenital heart diseases in newborns of mothers with GDM by evaluating the existence of a correlation with maternal glycemic control. This observational prospective study investigated newborns of mothers with GDM enrolled during a period of 9 months. Four subgroups were considered according to the type of maternal glucidic alteration during pregnancy and the home treatment: impaired glucose tolerance, insulin-dependent gestational diabetes mellitus (IDDM), non-insulin-dependent gestational diabetes mellitus (NIDDM), and gestational diabetes not controlled (NC: untreated diabetes). Student’s t test was used to compare the subgroups. The study enrolled 65 newborns (30 boys) born to 82 of mothers with impaired glucidic metabolism. Patent ductus arteriosus was observed in 11 patients (16.9 %), pulmonary stenosis of mild grade in 4 patients ( 6.2 %), and hypertrophy of the ventricular septum in 22 patients (33.8 %). A total of 14 patients had increased thickness in the left ventricle posterior wall, and 17 patients had an abnormal electrocardiogram. Hyperglycemia can influence the development of the fetal heart, affecting both its structure and its function. A treatment with insulin for women with GDM is supported by the study data.     

Risks of congenital anomalies in large for gestational age infants

OBJECTIVES: To evaluate the association between large for gestational age (LGA) and demographic and medical risk factors as well as specific types of congenital anomalies. STUDY DESIGN: A retrospective, case-control study on 2,149,617 consecutive births was conducted. LGA was defined as 1.64 SD above the mean weight for gestational age, adjusted by sex and altitude. Risk factor frequency distributions were compared between LGA and normal birth weight neonates. Associations between LGA and 41 infants with isolated congenital anomalies were evaluated. RESULTS: Of 31,897 neonates with congenital anomalies, 1800 were LGA. Five anomalies were associated with LGA: talipes calcaneovalgus, hydrocephaly, combined angiomatoses, hip subluxation, and non-brown-pigmented nevi. Multiparity, vaginal bleeding, diabetes, and delivery by cesarean section were more frequent in LGA than in appropriate for gestational age infants' mothers. Several maternal but no paternal factors were statistically associated with an increased risk for LGA infants. CONCLUSIONS: The clinical observation that nevi are more commonly observed in LGA patients was supported. The higher frequencies of hip subluxation and talipes calcaneovalgus among LGA neonates reinforces their pathogenesis as deformations, whereas those of combined angiomatoses and hydrocephaly could reflect increased fluid or body mass.     

Diabetes Mellitus in Friedreich's Ataxia

In a clinical study of Friedreich's ataxia diabetes mellitus was found in nine of 50 typical cases. The mean age of the diabetes onset was 21.9 years and the clinical type was a severe ketosis-prone diabetes. In 18 non-diabetics an intravenous glucose tolerance test was performed. One of these cases was disclosed to be a prediabetes. The mean rate of glucose disappearance was normal. Serum lipid analysis showed a slight elevation in non-diabetics and a higher in diabetics. The frequency of diabetes mellitus, including the prediabetic case, was 20%. Owing to the low age of the cases and as the glucose tolerance test was performed only in about half of the non-diabetics, this figure was estimated to be lower than the total frequency in Friedreich's ataxia. A brief survey of the literature is given, and the aetiology of diabetes mellitus in Friedreich's ataxia is discussed. The marked frequency of a known heredity to diabetes in the diabetic cases lends support to the hypthesis of a genetic aetiology. The possibility of an interplay between the defective genes of the two diseases is assumed. The pronouncedly vegetative syndrome occurring in Friedreich's ataxia suggests the possibility of a central nervous factor, though this can hardly be a primary cause of the diabetes.     

肥胖儿童伴良性黑棘皮病与胰岛素抵抗19例分析

目的　探讨肥胖儿童伴良性黑棘皮病与胰岛素抵抗及 2型糖尿病的关系。方法2 0 0 3年 6月～ 2 0 0 3年 9月 ,在我院内分泌门诊及病房就诊的体重指数 (BMI)≥ 2 5的肥胖儿童共 76例 ,对其中伴黑棘皮病皮肤改变的 19例 ( 2 5 % )均行皮肤病理活检以明确诊断 ,同时对这些患儿行空腹血糖、空腹血胰岛素水平、空腹血糖 /胰岛素比值 (FGIR)及人体测量学参数 [腰围 /臀围比值(WHR) ,全身体脂含量 (FM)、体脂百分数 (BF % )、体重指数 (BMI) ]等的检测 ,并行葡萄糖耐量试验(OGTT试验 ) ,以探讨肥胖儿童伴良性黑棘皮病与胰岛素抵抗及 2型糖尿病的关系。结果　 19例良性黑棘皮病患儿人体测量学参数包括腰围 /臀围比值 ,全身体脂含量 (FM)、体脂百分数 (BF % )、体重指数 (BMI)及空腹血胰岛素水平明显高于正常对照组 (P <0 0 1) ,空腹血糖 /胰岛素比值 (FGIR) ( 4 2 7± 0 5 3)小于 7,存在明显的胰岛素抵抗 ,其中 1例诊断为 2型糖尿病 ,10例有糖耐量异常。结论　儿童良性黑棘皮病与肥胖、高胰岛素血症 ,胰岛素抵抗及 2型糖尿病密切相关 ,是临床胰岛素抵抗的皮肤标志     

Gestational diabetes mellitus and glucose intolerance among Mexican pregnant adolescents

There is an increasing incidence of type 2 diabetes mellitus (DM) among adolescents (especially females), and the serum glucose concentrations in pregnant women <25 years during a 3-h oral glucose tolerance test (3-h OGTT) seem to be lower than those of pregnant women >25 years. Among 115 Mexican pregnant adolescents (<18 years) we analyzed their serum glucose concentrations during: a) 1-h 50-g glucose challenge test (GCT) performed at 24-28 weeks of gestation (n = 103) or at 29-35 weeks of gestation (n = 12); b) A standard 3-h OGTT performed 3-5 days later. Eight adolescents had an abnormal GCT, three of whom also had an abnormal 3-h OGTT. Sixteen adolescents (13 with previously normal GCT) had an abnormal 3-h OGTT, 15 classified as GGI and one as gestational DM (GDM). Serum glucose concentrations in adolescents with GGI were higher than in adolescents with normal 3-h OGTT: a) at 60 and 120 min during the 3-h OGTT (p < 0.001); and b) when expressed as the area under the glucose curve (p < 0.001). Adolescents with GGI had serum glucose concentrations during the 3-h OGTT similar to adult, non-diabetic pregnant Mexican women. It is suggested that GGI in pregnant adolescents may represent an early sign of a future deterioration in glucose metabolism, leading to a higher risk for GDM in future pregnancies and/or type 2 DM in adulthood. Thus, the current criteria to diagnose GDM in adults may not completely apply to adolescents, especially in ethnic groups with high risk for glucose abnormalities and considering the frequency of multiparous adolescents, especially in developing countries.     

Deaths among children less than two years of age receiving palivizumab: an analysis of comorbidities

BACKGROUND: Palivizumab (Synagis) is used for prophylaxis against respiratory syncytial virus infection among children at high risk for respiratory syncytial virus disease. A number of deaths after palivizumab use among children <2 years have been reported to the Food and Drug Administration. We assessed available information, including the extent to which preexisting medical conditions may have put these children at higher than normal risk of death. METHODS: We reviewed reports of deaths to the Food and Drug Administration (June 1998 to December 2001) among children <2 years of age who received palivizumab. RESULTS: There were 133 deaths reported after palivizumab use. Median age at death was 5 months, and 54% of the children were male. At least one congenital anomaly was reported in 85 cases (64%), and 44% of cases had multiple anomalies. Of the 100 cases with reported gestational age at birth, 36% were severely premature (<28 weeks), 48% were moderately premature (28 to 36 weeks) and 16% had normal gestational age. Only 2% of all cases were full term and were born without congenital anomalies; 50% had both conditions, 34% had prematurity alone and 14% had congenital anomalies alone. A cause of death was reported for 88 (66%) cases; most (38%) died from their congenital anomalies or from respiratory infections (23%). CONCLUSIONS: Most children dying after palivizumab treatment were at increased risk of death; many had multiple congenital anomalies and/or premature birth. Patterns of outcomes and the reported medical course did not suggest that palivizumab further elevated the risk of death. Current data do not alter the safety and efficacy assessment that led to the licensure of palivizumab.     

Obesity, gestational diabetes and pregnancy outcome

The prevalence of both obesity and gestational diabetes mellitus (GDM) is rising worldwide. The complications of diabetes affecting the mother and fetus are well known. Maternal complications include preterm labor, pre-eclampsia, nephropathy, birth trauma, cesarean section, and postoperative wound complications, among others. Fetal complications include fetal wastage from early pregnancy loss or congenital anomalies, macrosomia, shoulder dystocia, stillbirth, growth restriction, and hypoglycemia, among others. The presence of obesity among diabetic patients compounds these complications. The above-mentioned short-term complications can be mediated by achieving the desired level of glycemic control during pregnancy. However, GDM during pregnancy is associated with increased risk of early obesity, type 2 diabetes during adolescence and the development of metabolic syndrome in early childhood. Additionally, GDM is a marker for the development of overt type 2 diabetes and metabolic syndrome for the mother in the early future.     

Maternal nutritional status, diabetes and risk of macrosomia among Native Canadian women

Multivariate methods were used to identify risk factors for macrosomia (birth weight >4000 g) among 741 singleton births to Native Canadian women from Sioux Lookout Zone, Ontario, Canada, in 1990–1993. The average birth weight was 3691±577 g, and 29.2% of infants weighed more than 4000 g at birth. Macrosomic infants were born at later gestational ages and were more likely to be male. Women delivering macrosomic infants were taller, entered pregnancy with higher body mass indexes (BMI) and gained more weight during pregnancy, but were less likely to smoke cigarettes. They were more likely to have previously delivered a macrosomic infant and to have had gestational diabetes mellitus (GDM). Risk of macrosomia was associated with maternal glycemic status; women with pre-existing diabetes were at greatest risk, followed by those with GDM A₂ (fasting glucose ≥6 mmol/l). Women with GDM A₁ (fasting glucose <6 mmol/l) were not at increased risk for delivering a macrosomic infant, but glucose-tolerant women with high glucose concentrations 1 h after the 50 g challenge were at somewhat increased risk. Maternal glycemic status and maternal nutritional status before and during pregnancy are important determinants of macrosomia in this native population.     

THE SIGNIFICANCE OF ABNORMAL GLUCOSE TOLERANCE IN CHILDHOOD*

This paper refers to 13 children who were found to have glycosuria on routine testing of urine, but who had no symptoms referable to diabetes. Each child had one or more abnormal glucose tolerance tests on initial testing, but further observations revealed continuing glucose in only 1 child, and normal glucose tolerate in all those retested. The significance of abnormal glucose tolerance ni children without symptoms referabale to diabetes, is discussed. Treatment appropriate to diabetes mellitus is not recommended in the absence of clinical symptoms and with only the evidence of one abnormal glucose tolerance test. Further observations during succeeding months should first be made.     

Verbesserung der Glukosetoleranz von Patienten mit Zystischer Fibrose unter pseudomonaswirksamer Chemotherpie

Background. For many patients with cystic fibrosis impaired glucose tolerance or even diabetes mellitus is becoming relevant with growing age. The influence of an anti-Pseudomonas chemotherapy on glucose homeostasis of cystic fibrosis patients was investigated. Patients and methods. In fourteen cystic fibrosis patients aged between 7 and 35 years glucose tolerance was tested by standard oral glucose tolerance test in the beginning and at the end of a routine anti-Pseudomonas chemotherapy of fourteen days. Beside the blood glucose serum insulin was determinated. Results. According to the criteria of the American Diabetes Association three of the fourteen patients had an impaired glucose tolerance and another three had diabetes mellitus when tested at the beginning of anti-Pseudomonas chemotherapy. In four of these six patients glucose tolerance was normal at the end of the chemotherapy. Of the remaining two patients one fulfilled the criteria for impaired glucose tolerance and one for diabetes mellitus. In these patients insulin secretion was lower in the second test. Peak insulin was reached earlier while there was no significant improvement of early insulin response. Conclusion. The treatment of chronic airway infection in cystic fibrosis patients with impaired glucose tolerance or diabetes mellitus results in an improvement of glucose homeostasis by a better insulin sensitivity and less by improvement of early insulin response. In developing diagnostic protocols for screening of cystic fibrosis-related diabetes mellitus the impact of the concomitant therapy on glucose homeostasis should be considered.     

A case of severe congenital kyphoscoliosis secondary to multiple bilateral thoracic pedicle aplasia

A 9-month-old boy with congenital kyphoscoliosis secondary to multiple bilateral thoracic pedicle aplasias is presented. This anomaly has rarely been described in the literature. Plain films showed absence of thoracic pedicles bilaterally from T2 to T9, which was better demonstrated on CT multiplanar and 3-D reformatted images. There were no neurological symptoms even though the deformity progressed rapidly. Congenital kyphosis or kyphoscoliosis is often related to spinal anomalies that are located on the anterior aspect of the vertebrae. However, posterior anomalies may also be responsible and should be recognized before neurological complications occur.