Evaluation of developmental profiles of children with hydrocephalus / Evaluación de perfiles de desarrollo de niños con hidrocefalia

Objective The objective of this study was to compare the developmental characteristics of children with hydrocephalus with those of healthy children. Material and methods A total of 109 children aged between 2 and 46 months were included in the study, 54 patients diagnosed with hydrocephalus and 55 healthy children were evaluated with demographic data forms and Denver Developmental Screening Test II. Results The mean personal–social (p<0.001), fine motor-adaptive (p<0.001), language (p<0.001), and gross motor subscale scores were significantly lower in children with hydrocephalus than in the control group. Personal–social (p=0.002) and gross motor (p=0.029) subscale scores were significantly lower in children with obstructive hydrocephalus than communicating hydrocephalus. There was a significant negative correlation between language scores and ages of the children with hydrocephalus (r=−0.350, p=0.009). It was found that children with obstructive hydrocephalus carry a 6.7 folds higher risk of experiencing problems in terms of personal–social development compared to those with communicating hydrocephalus (p=0.011). Conclusion We found that patients with hydrocephalus were developmentally retarded compared to the healthy control subjects. Retardation was the most prominent in the obstructive group. Our results showed that neurodevelopmental follow-up should be carried-out regularly in pediatric patients with hydrocephalus, and early intervention should be started in necessary cases. (AU)

Objetivo El objetivo de este estudio fue comparar las características del desarrollo de niños con hidrocefalia con las de los niños sanos. Materiales y métodos Se incluyeron en este estudio un total de 109 niños con edades entre dos y 46 meses. Del total, 54 pacientes diagnosticados con hidrocefalia y 55 niños sanos fueron evaluados con formularios de datos geográficos y test screening de desarrollo Denver II. Resultados El promedio de los puntajes de las subescalas: desarrollo personal y social (p<0,001), motricidad fina-adaptativa (p=0,001), lengua (p=0,001) y motricidad gruesa fueron significativamente menores en niños con hidrocefalia que en el grupo control. Los puntajes de las subescalas desarrollo personal y social (p=0,002) y motricidad gruesa (p=0,029) resultaron significativamente menor en niños con hidrocefalia obstructiva que en hidrocefalia comunicante. Hubo una correlación negativa significativa entre los puntajes de lengua y las edades de los niños con hidrocefalia. (r=−0,350, p=0,009). Se encontró que los niños con hidrocefalia obstructiva portaban 6,7 veces un mayor riesgo de experimentar problemas en términos de desarrollo personal-social comparado con aquellos con hidrocefalia comunicante (p=0,011). Conclusión Encontramos que pacientes con hidrocefalia presentaron un retraso madurativo en comparación con los sujetos de control sanos. El retraso madurativo fue lo más prominente en el grupo obstructivo. Nuestros resultados mostraron que debería realizarse un seguimiento de neurodesarrollo regularmente en pacientes pediátricos con hidrocefalia y una intervención temprana debería comenzar en los casos que lo requieran. (AU)

The neurodevelopmental profile of healthy children with premature anterior fontanel closure.

BACKGROUND: We aimed to assess the neurodevelopmental status of healthy children with premature anterior fontanel closure. METHODS: This retrospective observational study was conducted on 40 (20 M, 20 F) children admitted to Mersin University Pediatric Neurology Outpatient Clinic between 2015-2020 with complaints of premature fontanel closure. Patients with dysmorphic features, microcephaly, craniosynostosis, hypoxic-ischemic sequelae, infections, metabolic disorders, intracranial hemorrhage, epilepsy, endocrine problems, additional congenital anomalies, intrauterine growth retardation (IUGR), prematurity, and postmaturity were excluded. The Denver II and Bayley III tests were applied to all patients and controls. RESULTS: The Denver II identified retardations in gross motor skills (p = 0.015) and personal-social skills (p = 0.042) and Bayley III in cognitive (p = 0.030) and motor skills (p = 0.007) in the study group. None of the participants in the study group had neurodevelopmental retardation, according to the Bayley III normal standards. DISCUSSION: Our results suggest that children with premature fontanel closure may develop motor retardation. These children should, therefore, be closely monitored for neurodevelopmental aspects.

Evaluation of developmental profiles of children with hydrocephalus.

OBJECTIVE: The objective of this study was to compare the developmental characteristics of children with hydrocephalus with those of healthy children. MATERIAL AND METHODS: A total of 109 children aged between 2 and 46 months were included in the study, 54 patients diagnosed with hydrocephalus and 55 healthy children were evaluated with demographic data forms and Denver Developmental Screening Test II. RESULTS: The mean personal-social (p<0.001), fine motor-adaptive (p<0.001), language (p<0.001), and gross motor subscale scores were significantly lower in children with hydrocephalus than in the control group. Personal-social (p=0.002) and gross motor (p=0.029) subscale scores were significantly lower in children with obstructive hydrocephalus than communicating hydrocephalus. There was a significant negative correlation between language scores and ages of the children with hydrocephalus (r=-0.350, p=0.009). It was found that children with obstructive hydrocephalus carry a 6.7 folds higher risk of experiencing problems in terms of personal-social development compared to those with communicating hydrocephalus (p=0.011). CONCLUSION: We found that patients with hydrocephalus were developmentally retarded compared to the healthy control subjects. Retardation was the most prominent in the obstructive group. Our results showed that neurodevelopmental follow-up should be carried-out regularly in pediatric patients with hydrocephalus, and early intervention should be started in necessary cases.

Developmental evaluation in children experiencing febrile convulsions.

BACKGROUND: The objective of this study was to determine the effect of febrile convulsion (FC) on neuromotor development. METHODS: Data of 325 patients, who were followed up at our outpatient clinic and diagnosed with FC between January 2012 and December 2018, were retrospectively evaluated. Of these patients, 203 underwent the Denver Developmental Screening Test II (DDST II) and were included in the study as the patient group and 100 healthy children as the control group. RESULTS: Of the study group, 84 (41.4%) were girls and 119 (58.6%) were boys (B/G: 1.4). Of all patients, 163 (80.3%) were diagnosed with simple FC, 22 (10.8%) with complicated FC, and 18 (8.9%) with FC+. There was no significant relationship found between FC subtypes and gender, family history of FC, family history of epilepsy, iron (Fe) deficiency, and Fe deficiency anemia. DDST II subtest points were significantly lower in all developmental areas in the patient group when compared to the controls (p < 0.001), while suspected and abnormal test results were higher in all developmental areas in the patient group compared to the controls (p=0.01). It was also determined that the language points were lower as the age of first seizure increased (r=- 0.319, p < 0.01). CONCLUSIONS: Although FC is known to usually having a good prognosis, the low DDST II test results measured in this study indicated that the FC may pose a developmental risk and patients with FC should be followed up in terms of developmental features. Because of the retrospective nature of the study, there was no `preconvulsion` developmental evaluation. This is a major limitation of our study.

Long-term cognitive outcomes of prematurely born infants: A longitudinal follow-up of Denver II, Bayley III and WISC-IV Tests.

BACKGROUND: Prematurity constitutes a risk factor for developmental delay in infancy and childhood. This study aims to: (i) determine long-term cognitive outcomes in prematurely delivered children and compare them with term-delivered children using the WISC-IV and Stroop tests; (ii) examine the relation between Denver II, Bayley III and WISC-IV, Stroop tests. METHODS: The study group consisted of children born prematurely who had been tested with Denver II and Bayley III in their first 2 years, and had been evaluated with WISC-IV and Stroop tests under follow up, 6-10 years later. RESULTS: The study group (n = 60, 25 F, 35 M) was 8.0 ± 2.4 (6-10.7) years old when given WISC-IV and Stroop tests. Gestational age in the study group was 34-37 weeks in 25%, 30-33 weeks in 48.3%, and <29 weeks in 26.7%. On WISC-IV, the verbal comprehension index, perceptual reasoning index, working memory index, and full-scale IQ scores were lower in the study group than the control group (P < 0.05). The study group took longer to complete the Stroop test (P < 0.05). Lower socioeconomic status (P = 0.005) and parental education level (P = 0.000) were associated with lower verbal comprehension index scores. Denver II and Bayley III test results were related to WISC-IV results (P < 0.05) but not to the Stroop test results (P > 0.05). CONCLUSIONS: Our results showed prematurity negatively influences the results of WISC-IV and Stroop tests at school age. Denver II and Bayley III tests applied at age 2 years likely predict WISC-IV results.

Neurodevelopmental risk evaluation of premature closure of the anterior fontanelle.

PURPOSE: To evaluate neurological development of completely healthy children with anterior fontanelle premature closure via Denver Developmental Screening Test II and to compare the results with control group. METHOD AND RESULTS: The records of 140 patients applied to Mersin University Pediatric Neurology Outpatient Clinic between 2011 and 2019 with the complaint of premature closure of the anterior fontanelle were retrospectively reviewed. Patients with microcephaly, craniosynostosis, infection, sequelae of hypoxia-ischemia, metabolic disorders, intracranial hemorrhage, epilepsy, endocrine problems, and dysmorphic features were excluded from the study. Sixty-six completely healthy children with anterior fontanelle premature closure were included in the study. Denver Developmental Screening Test II was performed by the same developmental specialist to the children with premature closure of the anterior fontanelle as well as to the healthy control group. For each child included in the case and the control group, 90% of the values for each development area were calculated and recorded. Then, the results were compared. Denver II Developmental Screening Test (p < 0.001) and gross motor subtest (p < 0.001) results showed statistically significant retardation in the case group compared with the control group. CONCLUSIONS: The study was the first study in the literature on the gross motor development of children with premature closure of anterior fontanelle, and it has been found significantly undeveloped compared with the control group, and it has been concluded that similar patients should be evaluated from this view point in pediatric neurology department.

Comparison of selective head cooling versus whole-body cooling.

BACKGROUND: This study compared selective head cooling (SHC) and whole-body cooling (WBC) in newborns with hypoxic-ischemic encephalopathy (HIE). METHODS: We conducted a prospective randomized small-scale pilot study in newborns with HIE, born after >35 weeks of gestation. The patients were randomly assigned to receive SHC or WBC. RESULTS: The SHC group consisted of 17 patients, and the WBC group, 12 patients. There was no significant difference in adverse effects related to cooling therapy between the two groups. During the 12 month study period, seven patients in the SHC group and four in the WBC group died, but the difference was not significant (P = 0.667). Among the patients alive at 12 months after treatment, six in the SHC group and four in the WBC group had severe disabilities; the difference was not significant (P = 0.671). When the composite outcome of death or severe disability was evaluated, the difference between the SHC group (77%, n = 13) and the WBC group (67%, n = 8) was not significant (P = 0.562). Moreover, the number of survivors without disability at 12 months after treatment did not differ significantly between the SHC group (n = 3) and the WBC group (n = 4; P = 0.614). CONCLUSIONS: There were no significant differences in adverse effects, 12 month neuromotor development, or mortality rate between SHC and WBC in newborns with HIE, born after >35 weeks of gestation.

The effects of selective head cooling versus whole-body cooling on some neural and inflammatory biomarkers: a randomized controlled pilot study.

BACKGROUND: Therapeutic hypothermia (TH) has become standard care in newborns with moderate to severe hypoxic ischemic encephalopathy (HIE), and the 2 most commonly used methods are selective head cooling (SHC) and whole body cooling (WBC). This study aimed to determine if the effects of the 2 methods on some neural and inflammatory biomarkers differ. MATERIALS AND METHODS: This prospective randomized pilot study included newborns delivered after >36 weeks of gestation. SHC or WBC was administered randomly to newborns with moderate to severe HIE that were prescribed TH. The serum interleukin (IL)-1ß, IL-6, neuron-specific enolase (NSE), brain-specific creatine kinase (CK-BB), tumor necrosis factor-alpha (TNF-α), and protein S100 levels, the urine S100B level, and the urine lactate/creatinine (L/C) ratio were evaluated 6 and 72 h after birth. The Bayley Scales of Infant and Toddler Development-III was administered at month 12 for assessment of neurodevelopmental findings. RESULTS: The SHC group included 14 newborns, the WBC group included 10, the mild HIE group included 7, and the control group included 9. All the biomarker levels in the SHC and WBC groups at 6 and 72 h were similar, and all the changes in the biomarker levels between 6 and 72 h were similar in both groups. The serum IL-6 and protein S100 levels at 6 h in the SHC and WBC groups were significantly higher than in the control group. The urine L/C ratio at 6 h in the SHC and WBC groups was significantly higher than in the mild HIE and control groups. The IL-6 level and L/C ratio at 6 and 72 h in the patients that had died or had disability at month 12 were significantly higher than in the patients without disability at month 12. CONCLUSION: The effects of SHC and WBC on the biomarkers evaluated did not differ. The urine L/C ratio might be useful for differentiating newborns with moderate and severe HIE from those with mild HIE. Furthermore, the serum IL-6 level and the L/C ratio might be useful for predicting disability and mortality in newborns with HIE.

Evaluation of Neurodevelopment Using Bayley-III in Children with Cyanotic or Hemodynamically Impaired Congenital Heart Disease.

OBJECTIVE: The purpose of this study was to compare neurological development of children with cyanotic or hemodynamically impaired congenital heart disease (CHD) and healthy controls by using "Bayley Scales of Infant and Toddler Development Screening Test, Third Edition" (Bayley-III). PATIENTS: Children with CHD (n = 37) and healthy controls (n = 24) aged between 1 and 41 months who were admitted to the Department of Pediatric Cardiology at our university hospital were included. The participants were assessed using Bayley-III test. All patients had cyanotic or hemodynamically impaired CHD. Weight, height, body mass index (BMI), mid-arm circumference (MAC), triceps skinfold thickness (TSF), and head circumference (HC) were measured and standard deviation scores (SDSs) were determined. RESULTS: SDS values of weight, height, BMI, MAC, and TSF of the patients as well as HC values were significantly lower than the control group (P < .001). Compared with controls, the patients had significantly lower mean scores in all Bayley-III subscales (P < .001). We observed similar results in Bayley-III scores including the mean values of cognitive, language, and global motor scores for the CHD patients with and without cardiac surgery (P > .05). CONCLUSION: This study demonstrated that children with cyanotic or hemodynamically impaired CHD have delayed neurodevelopmental outcomes compared with healthy children as assessed using Bayley-III.

Consistency between referral diagnosis and post-ENMG diagnosis in children.

OBJECTIVE: To evaluate the degree of consistency between the referral diagnosis and that based on electroneuromyography. METHODS: The retrospective study was conducted at the Paediatric Neurology Laboratory of Mersin University School of Medicine, Turkey, and comprised all electroneuromyographies carried out between January 2005 and December 2010. Demographic data, referral diagnosis and post-procedure diagnosis were recorded for each patient, and were classified into groups. Consistency between the two groups was compared using SPSS 13. RESULTS: Of the total 294 patients, polyneuropathy was the reason for referral in 104 (35.4%), peripheral nerve injury in 54 (18.4%), brachial plexus injury in 52 (17.7%), myopathy in 52 (17.7%), hypotonia in 23 (7.8%), and facial paralysis in 9 (3.0%) patients. There was consistency between the two diagnoses in 179 (60.9%) patients. CONCLUSION: Electroneuromyography is an uneasy, painful and stressfull procedure for children, and, therefore, it should be recommended only in cases where the result may be beneficial in the diagnosis treatment and follow-up of a patient.

Neurodevelopment evaluation in children with congenital hypothyroidism by Bayley-III.

BACKGROUND: Congenital hypothyroidism is the most common reason of mental retardation, and normal neurological development can be provided by early and effective treatment. In this present study, it is aimed to compare neurological developments of patients in 6-42 months of age with congenital hypothyroidism and healthy controls of the same age group prospectively by Bayley III test. METHODS: In this present study, neurological developments of 41 congenital hypothyroidism cases and 39 healthy controls, who applied to Pediatric Endocrinology Section of Mersin Children Hospital and Pediatric Neurology Outpatient Clinic of the Medical School at Mersin University between years 2009 and 2011, were evaluated by Bayley III test. RESULTS: Cognitive, language and global motor scores in addition to receptive communication, expressive communication, fine motor and gross motor subscores in children with congenital hypothyroidism were statistically significantly lower than those in the control group (p<0.05). It is detected that initiation dose and day of treatments, severity of hypothyroidism and time to normalization thyroid stimulating hormone had no statistically significant effects on neurological development of the study group (p>0.05). In both groups, as the education levels of mothers are increased, language development scores are also increased (p<0.05). Additionally, statistically significant increases in Bayley III scores except cognitive scores have been observed in both groups as the level of income is increased (p<0.05). CONCLUSIONS: Despite early and effective treatment in newborns with congenital hypothyroidism, retardation in neurological developmental has been detected. This situation can be related to influences on neurodevelopment in intrauterine period. According to our present knowledge, this study is the first case-control study in the literature that neurological developments of congenital hypothyroidism patients are evaluated with Bayley-III score.

Evaluation of growth and neurodevelopment in children with congenital heart disease.

BACKGROUND: Children with congenital heart disease are under risk of delayed growth and development. We evaluated physical growth parameters and neurodevelopment in these patients in comparison with normal children and examined the effect of hemodynamic status. METHODS: Patients with congenital heart disease (n= 76) and healthy children (n= 51) aged 1-72 months applied to Mersin University Hospital, Mersin, Turkey were included. Patients with heart failure and those requiring intervention or surgery were classified as hemodynamically impaired (HI group, n= 30), and the others, hemodynamically normal (HN group, n= 46). Growth parameters including weight, height, body mass index (BMI), mid-arm circumference (MAC), and triceps skin fold thickness (TSF) were measured and standard deviations (SD) were determined. Functional development was assessed by Denver Developmental Screening Test-II (DDST II). RESULTS: MAC and BMI values of the group with impaired hemodynamic status were significantly lower than the hemodynamically normal and control groups (MAC P < 0.05 and BMI P < 0.01). In the DDST II, the group with hemodynamic abnormality had more failures in gross motor and fine motor skills than HN group and controls (gross motor P= 0.011, P < 0.001 and fine motor P= 0.028, P= 0.001, respectively) and more failures in language development than the control group (P= 0.001). CONCLUSION: The results showed the importance of hemodynamic status in growth and neurodevelopment of children with congenital heart disease. Besides routine growth parameters, more detailed examinations such as BMI, MAC, TSF, and developmental screening tests appear useful in identifying children with cardiac disease who are under risk for delayed growth and development.

Effects of antiepileptic drug therapy on heart rate variability in children with epilepsy.

Impaired cardiac autonomic function may contribute to the risk of sudden unexpected death in epilepsy. Heart rate variability (HRV) is a useful tool for the detection of sympathetic-parasympathetic balance of autonomic nervous system. In the present study, epilepsy patients who had never received antiepileptic medication and those whose seizures have been successfully controlled with antiepileptic drugs were compared with each other and a control group in order to investigate the effects of epilepsy and various antiepileptic drugs on HRV. HRV were tested via 5 min ECG monitoring in 92 patients and 83 controls. Time domain parameters including SDNN, RMSSD and the frequency domain parameters including HF (reflects parasympathetic activity) and LF (reflects sympathetic activity) were assessed. In this group, 78 patients were using antiepileptic drugs including valproic acid (n=33), oxcarbazepine (n=19), phenobarbital (n=11), combined regimens (n=10) and other drugs (n=5), while 14 patients had never received antiepileptic medication. For both of the epilepsy patients groups with or without treatment, time domain parameters were found to be significantly suppressed. In addition, parasympathetic activity was found to be decreased (HF was decreased, LF/HF ratio was increased) in epilepsy patients without antiepileptic drug therapy. Our results indicate that seizure control with antiepileptic drugs may help to improve the cardiac autonomic function impairment in epilepsy patients.