Changes in parents' attitudes towards childhood vaccines during COVID-19 pandemic.

BACKGROUND: Concerns about the safety and adverse reactions of rapidly-developed vaccines against COVID-19 contributed to parents' vaccine hesitancy and this situation created an opportunity for anti-vaccine campaigners. The aim of this study was to examine the changes in parents' attitudes towards childhood vaccines during COVID-19 pandemic. METHODS: In this cross-sectional study, parents of children who applied to the outpatient clinic of pediatric department of Trakya University Hospital, Edirne, Türkiye, between August 2020 and February 2021 were recruited into two study groups according to COVID-19 peak time in Türkiye. Group 1 included parents of children who applied after first peak of the COVID-19 pandemic and Group 2 included parents of children who applied after second peak. The World Health Organization 10-item Vaccine Hesitancy Scale (WHO-VHS) was applied to each group. RESULTS: A total of 610 parents agreed to participate in the study. Group 1 and 2 consisted of 160 and 450 parents, respectively. While the number of parents who were hesitant about childhood vaccines was 17 (10.6%) in Group 1, it was 90 (20%) in Group 2. A statistically significant difference was found between the two groups (p = 0.008). The (WHO-VHS) mean ± SD score was found to be higher in Group 2 (23.7 ± 6.9) than Group 1 (21.3 ± 7.3) (p < 0.001). The WHO-VHS- mean ± SD scores of parents who experienced COVID-19 infection themselves or their family or acquaintances were significantly lower than those who did not (20.0 ± 6.5 vs. 24.7 ± 6.9) (p < 0.001). CONCLUSIONS: The hesitant attitudes towards childhood and COVID-19 vaccines were low in parents who directly or indirectly experienced COVID-19 or were worried about the devastating effects of this disease. However, it was shown that as the COVID-19 pandemic progressed, parents' hesitations towards childhood vaccines increased.

Eating Behaviors of Late and Moderately Preterm Infants at Two Years of Age and Their Associations With Mothers' Mental Health.

OBJECTIVES: Preterm infants are at high risk for nutritional difficulties during the neonatal period and early childhood. Long-term nutritional difficulties contribute to unbalanced food intake and growth disorders and are the source of major emotional stress for the family. The aim of this study is to investigate the eating problems of late and moderately preterm (LMPT) infants at the age of 2 years, and to examine the association of these problems with the mental status of their mothers. METHODS: In this cross-sectional study, group 1 included LMPT infants born between 32 and 36 + 6 weeks of gestation and Group 2 included term infants born between 37 and 41 + 6 weeks of gestation. Children's Nutrition Difficulties Questionnaire and 21-item Depression Anxiety and Stress Scales (DASS 21) were used for the detection of nutritional difficulties of infants and mothers' mental health status. RESULTS: Groups 1 and 2 were consisted of 79 LMPT and 38 term infants, respectively. Late and moderately preterm infants were found to have a lower drive-to-eat and food repertoire scores as well as lower appetite and food enjoyment than term infants. Pickiness and food neophobia were found to be higher in LMPT infants than term infants. The DASS-21 scores of the mothers of the LMPT infants were higher than those of the term ones. CONCLUSIONS: These findings suggest that LMPT infants have more nutritional difficulties at the age of 2 years than term infants and their mothers exhibit more emotional distress than term infant's mothers.

[Meningococcemia: Different Serotypes in the Same Region]. / Meningokoksemi: Ayni Cografyada Farkli Serotipler.

Meningococcal infections are important health problems causing high morbidity and mortality. Neisseria meningitidis have 13 serogroups. A, B, C, Y and W135 are the most common causes of invasive disease among those serogroups. The distribution of the serogroups differs according to the geographical regions and the age groups. In this case report, two cases of meningococcemia infected with serogroup C and Y of N.meningitidis rarely seen in our country were presented. First case was a two and a half year-old female patient who has admitted to our pediatric emergency unit with fever and rash spreading from lower extremities to her body. The patient had diffuse purpuric rash with generalized weakness and tendency to sleep at admission. The patient has been suspected as meningococcemia because of the skin rash, tendency to sleep and hypotension. Antibiotics treatment was started immediately and lumber puncture was performed. In blood tests, leukocyte count: 3600/mm3 (61% neutrophils), hemoglobin: 11.1 g/ dl, platelet count: 127.000/mm3 , C-reactive protein: 10 mg/dl, erythrocyte sedimentation rate: 6 mm/ hour, prothrombin time: 28.8 seconds (normal value= 11-16), prothrombin activity: 36%, international normalized ratio (INR): 2.13 (normal value= 1-1.5), activated partial thromboplastin time: 57.7 seconds (normal value= 25-35 sec), fibrinogen: 246 mg/dl (normal value= 200-400 mg/dl) and in cerebrospinal fluid protein: 21 mg/dl and glucose: 62 mg/dl were found. There were eight cells in the microscopic examination. Skin rashes were increased and the patient became hypotensive. No microorganisms were isolated in blood and cerebrospinal cultures. N.meningitidis serogroup C was isolated from the cerebrospinal fluid of the patient using polymerase chain reaction (PCR). The patient suffered from immune-mediated arthritis in the sixth day of treatment and nonsteroidal anti-inflammatory drugs were given. The patient has recovered with antibiotics, fresh frozen plasma and inotropic treatment. Second case was a 13 year-old male patient who has admitted three days after the first case with a pre-diagnosis of malignancy because of pancytopenia and fever. The patient had generalized weakness and a few petechial purpuric rashes at the facial region at admission. After the admission general status of the patient has worsened rapidly and he has died as a result of cardiovascular arrest. Blood tests in admission showed leukocyte count: 6000/mm3 (79% neutrophils), hemoglobin: 17.3 mg/dl, platelet count: 16.000/mm3 , C-reactive protein: 8.63 mg/dl, prothrombin time: 92.6 seconds, prothrombin activity: 10%, INR: 6.78, activated partial thromboplastin time: 231.5 seconds. Cerebrospinal fluid obtained from postmortem lumbar puncture showed no growth (protein: 95 mg/dl, glucose: 35 mg/dl) and N.meningitidis serogroup Y was detected by PCR. Two meningococcemia cases caused by two different serogroups which are rarely seen in our region in recent years were presented at the same time period in the same hospital. This case report pointed out that surveillance has a great importance in such diseases.

Effect of neonatal resuscitation courses on long-term neurodevelopmental outcomes of newborn infants with perinatal asphyxia.

BACKGROUND: In previous studies, it has been demonstrated that Neonatal Resuscitation Program (NRP) courses improve the early outcomes of infants with perinatal asphyxia, but there has been no evidence to demonstrate the effect of NRP on long-term outcomes of perinatal asphyxia. The goal of the present study was to determine the effect of NRP courses on the long-term neurodevelopmental outcome of perinatal asphyxia. METHODS: This prospective study included infants referred to the Neonatal Unit during the years 2003-2005. Those patients who were referred before NRP courses (pretraining period) were designated as group 1, those who were referred after the first NRP course (transition period) as group 2, and those who were referred after the second NRP course (post-training period) as group 3. Neurodevelopmental outcomes were assessed and compared at 4-6 years of age. RESULTS: The study involved 40 patients: 23 in group 1, nine in group 2 and eight in group 3. The number of patients who had been diagnosed with cerebral palsy was 13 in group 1, two in group 2, and one in group 3, which was a significant decrease. The number of patients with seizures and electroencephalography abnormality was 12 and 14 in group 1, three and two in group 2, and one and one in group 3, respectively, which was also a significant decrease. CONCLUSIONS: NRP courses have positive effects on short-term as well as long-term neurodevelopmental outcomes of infants with perinatal asphyxia. Further studies are required to determine the effects of NRP courses on minor deficits, such as cognitive and behavioral disturbances.

De Novo Subtelomeric 6p25.3 Deletion with Duplication of 6q23.3-q27: Genotype-Phenotype Correlation.

Duplications of 6q and deletions of 6p have been reported in more than 30 cases of live born infants and given rise to widespread abnormalities recognizable as a specific clinical syndrome. Different phenotypes have been described with variable clinical signs. Most cases involve the coexistence of unbalanced translocations affecting one or the other of the chromosomes. However, duplication of both chromosome 6q and deletion of 6p regions have been reported in only a few cases. Here, we report the first duplication of chromosome band 6q23.3-q27 with deletion of 6p25.3. This is the first case in the literature involving changes to these specific chromosomal regions; a medium size duplication of the distal long arm and smaller deletion of the terminal short arm of chromosome 6. In the literature, there are no other cases where these two specific chromosomal aberrations are observed together. Conventional chromosome analysis was performed to investigate the patient. Chromosome structure was identified using fluorescence in situ hybridization for subtelomeric regions of chromosome 6 and array comparative genomic hybridization analysis (array-CGH).

Comparison of sleep problems between term and preterm born preschool children.

OBJECTIVES: Sleep disorders are common problems among all age groups. If sleep problems detected in childhood are not resolved successfully, they become sleep disorders and a chronic state. In this study, we aimed to compare sleep problems between preterm-born and term-born preschool-age children. METHODS: In the cross-sectional study, children were grouped according to whether they were born preterm or at term. The preterm group included 137 children aged four to six years. The control group comprised 145 age-matched term-born preschool children. The prenatal, natal, demographical, and clinical characteristics of preterm- and term-born preschool-age children were compared. The Children's Sleep Habits Questionnaire (CSHQ) was used to identify sleep problems. The total score and subscores of the CSHQ were compared between the groups. RESULTS: The percentage of gastroesophageal reflux (GER) symptoms and obstructive sleep apnea (OSA) symptoms were significantly higher in the preterm group. According to the cutoff point of the CSHQ, 97 children in the preterm group (70.8%) and 88 children in the control group (60.7%) had a sleep disorder. The total score of the CSHQ was significantly higher in the preterm group compared with the control group; however, the scores of the subscales were similar between the groups. In the regression analysis, a significant association was found between being born preterm and having sleep disorder (ß = 0.308, OR = 1.36, p = 0.04). CONCLUSIONS: Our study reported a high percentage of sleep problems in preterm-born preschool children. We suggest that prematurity is associated with sleep problems even if the etiology of sleep problems is heterogeneous. Symptoms regarding GER and OAS should be investigated, and precautions, such as prohibiting maternal cigarette smoking, should be taken in preterm infants.

Comparison of temporal artery, mid-forehead skin and axillary temperature recordings in preterm infants <1500 g of birthweight.

AIM: Preterm infants are prone to temperature maintenance problems due to immature thermoregulatory mechanism and relatively large body surface area. The objective of the present study was to evaluate the performance of a new non-invasive infrared thermometer applied to the mid-forehead and temporal artery in comparison with axillary temperature recordings by mercury-in-glass thermometer, and to determine the discomfort caused by these procedures in preterm infants on incubator care. METHODS: The present comparative prospective study was composed of 34 preterm infants <1500 g of birthweight nursed in an incubator. Temperature recording from mid-forehead, temporal artery and axilla were recorded six times a day for 7 days since the end of the first week of life. For pain assessment, the premature infant pain profile (PIPP) was used. RESULTS: The mean mid-forehead, temporal artery and axillary temperatures were 36.72 +/- 0.08, 36.81 +/- 0.09 and 36.71 +/- 0.07 degrees C, respectively. No statistically significant difference was noted between the means of mid-forehead and axillary temperatures. The mean temporal artery temperature was statistically higher than the means of the mid-forehead and axillary temperatures. The PIPP scores of the mid-forehead, temporal artery and axillary temperature measurements were 5.07 +/- 0.36 degrees C, 5.18 +/- 0.43 degrees C and 7.59 +/- 0.84 degrees C, respectively. The mean PIPP score of axillary temperature measurements was statistically higher than the means of mid-forehead and temporal artery measurements. CONCLUSIONS: The infrared skin thermometer applied to the mid-forehead is a useful and valid device for easy and less painful measurement of skin temperature in preterm infants <1500 g of birthweight.

Early neonatal outcomes of very-low-birth-weight infants in Turkey: A prospective multicenter study of the Turkish Neonatal Society.

OBJECTIVE: To investigate the early neonatal outcomes of very-low-birth-weight (VLBW) infants discharged home from neonatal intensive care units (NICUs) in Turkey. MATERIAL AND METHODS: A prospective cohort study was performed between April 1, 2016 and April 30, 2017. The study included VLBW infants admitted to level III NICUs. Perinatal and neonatal data of all infants born with a birth weight of ≤1500 g were collected for infants who survived. RESULTS: Data from 69 NICUs were obtained. The mean birth weight and gestational age were 1137±245 g and 29±2.4 weeks, respectively. During the study period, 78% of VLBW infants survived to discharge and 48% of survived infants had no major neonatal morbidity. VLBW infants who survived were evaluated in terms of major morbidities: bronchopulmonary dysplasia was detected in 23.7% of infants, necrotizing enterocolitis in 9.1%, blood culture proven late-onset sepsis (LOS) in 21.1%, blood culture negative LOS in 21.3%, severe intraventricular hemorrhage in 5.4% and severe retinopathy of prematurity in 11.1%. Hemodynamically significant patent ductus arteriosus was diagnosed in 24.8% of infants. Antenatal steroids were administered to 42.9% of mothers. CONCLUSION: The present investigation is the first multicenter study to include epidemiological information on VLBW infants in Turkey. Morbidity rate in VLBW infants is a serious concern and higher than those in developed countries. Implementation of oxygen therapy with appropriate monitoring, better antenatal and neonatal care and control of sepsis may reduce the prevalence of neonatal morbidities. Therefore, monitoring standards of neonatal care and implementing quality improvement projects across the country are essential for improving neonatal outcomes in Turkish NICUs.

The impact of Neonatal Resuscitation Program courses on mortality and morbidity of newborn infants with perinatal asphyxia.

OBJECTIVE: Neonatal care provided within the first few minutes of life plays a major role in the reduction of neonatal morbidity and mortality. Neonatal Resuscitation Program (NRP) courses had been held since 1996. The aim of this study was to evaluate the impact of the NRP on morbidity and mortality of newborn infants with perinatal asphyxia. METHODS: This retrospective study comprised newborn infants who were born in hospitals at Trakya region of Turkey during the last 3 years and were diagnosed as perinatal asphyxia and were referred to our Neonatal Unit. Those patients who were referred before NRP course (pretraining period) were designated as Group 1, those who were referred after the first NRP course (transition period) as Group 2, and those who were referred after the second NRP course (post-training period) as Group 3. Chart review was performed with regard to gestational age, birth weight, Apgar scores, resuscitation type, stage of hypoxic ischemic encephalopathy (HIE), existence of meconium aspiration syndrome (MAS), progress of the disease, duration of hospitalization. RESULTS: The study comprised 66 patients; 35 in Group 1, 18 in Group 2 and 13 in Group 3. The number of cases who had not been resuscitated was 10 in the pretraining period, 3 in the transition period and 1 in the post-training period which decreased significantly. The first minute Apgar scores in three groups were as follows; 2.08+/-1.2, 2.2+/-1.1 and 3.7+/-1.4, and this increase was statistically significant. The fifth minute Apgar scores also increased from 5.43+/-1.5 in the pretraining period to 6.5+/-1.9 in the post-training period, but this increase was not statistically significant. The number of patients with Stage 1 and 2 HIE decreased more in Group 3 (n=11 in Stage 1 HIE, n=17 in Stage 2 HIE) compared to those in Group 1 (n=7 in Stage 1 HIE, n=5 in Stage 2 HIE) but the difference was not statistically significant. The duration of hospitalization decreased in post-training period (15.1+/-10.3 days in pretraining period, 12.0+/-8.9 days in transition period, 6.1+/-1.2 days in post-training period). CONCLUSIONS: After NRP courses, the number of patients with perinatal asphyxia and with no resuscitation and also the duration of hospitalization decreased significantly, whereas the first minute Apgar scores increased significantly.

Proficiency and knowledge gained and retained by pediatric residents after neonatal resuscitation course.

BACKGROUND: In previous studies the efficacy of the Neonatal Resuscitation Program (NRP) was evaluated, demonstrating good retention of knowledge in the participants. The aim of the present study was to evaluate the knowledge and proficiency that pediatric residents gained and retained following NRP and to determine the necessity and timing of the refresher courses. METHODS: The study consisted of 42 pediatric residents who were divided into three groups. Group 1 included pediatric residents who attended the course 1 year previously (n = 18); group 2, 6 months previously (n = 12); and group 3 included those residents who had not attended the NRP previously (n = 12). A written test consisting of 100 questions was applied, and endotracheal intubation skill on a neonatal manikin was evaluated. RESULTS: The percentage of correct answers of participants in groups 1 and 2 decreased similarly during the 6 month and 1 year time period following previous courses. After the new NRP the correct answers increased significantly in groups 1 and 2. The duration of intubation attempt was 17.8 +/- 6.0 s in group 1, 17.5 +/- 1.5 s in group 2, and 22.3 +/- 2.6 s in group 3 before the course and decreased after the course to 10.9 +/- 2.0 s in group 1, 10.3 +/- 1.5 s in group 2, and 11.7 +/- 1.6 s in group 3. The knowledge missing after 6 months and 1 year were similar, while intubation skill decreased after 1 year following the course. CONCLUSIONS: Due to worsening of test findings at 6 months after NRP, and deterioration of performance of intubation 1 year after NRP, it is suggested that a refresher course may be required every year.

Turkish Neonatal Society guideline for the follow-up of high-risk newborn infants.

Developments in perinatal and neonatal care have increased the survival rate of high-risk newborns but led to a rise in chronic diseases seen in these infants. A significant number of them attend primary and secondary health care centers after discharge; however, there are very few standard protocols for the long-term follow-up of these babies. Therefore, we aimed to establish a follow-up guideline that emphasizes on universal screening schemes and takes into consideration national data. The guide presented here provides brief recommendations for physicians in light of evidence-based data for the follow-up of high-risk newborn infants. The steps taken to monitor and solve the problems of all high-risk infants may vary. We hope the use of such a standard approach in evaluating each infant in daily routine will improve the life quality of these high-risk infants.

Diagnostic and therapeutic approach in newborns with ambiguous genitale with disorder of sex development: consensus report of Turkish Neonatal and Pediatric Endocrinology and Diabetes Societies.

Disorders of sex development are defined as conditions in which the chromosomal, gonadal, and anatomic sex is discordant. Patients usually present with atypical appearing genitalia. In the assessment of neonates with disorders of sex development, first, it is important to determine whether this situation requires prompt evaluation, and then the karyotype, hormone levels, and underlying etiology should be determined as soon as possible. All these procedures should be performed in the guidance of a multidisciplinary team in reference centers. As the physical examination of the infant is extremely important, the physcian should suspect and then perform a detailed history and physical examinationi and lastly plan the required laboratory and imaging procedures for the definite diagnosis. It is important not to be hurried in the choice of sex. The aim of this article, which includes the diagnostic and therapeutic approaches in infants with ambiguous genitalia, was to provide a common practice for all pediatricians.

Cranial MR venography findings of severe hypernatremic dehydration in association with cerebral venous thrombosis in the neonatal period.

Severe neonatal hypernatremia is an important electrolyte disorder that has serious effects on the central nervous system, including brain edema, intracranial hemorrhage, hemorrhagic infarct, and thrombosis. Cerebral venous thrombosis is relatively rare in severe neonatal hypernatremic dehydration. The English literature contains only a few reports of the cranial radiological findings in severe neonatal hypernatremia. The authors report cranial MR venography findings of a newborn infant with severe hypernatremic dehydration. To the best of their knowledge, this is the first such report in the English literature.

Factor V Leiden mutation, deficiency of antithrombin III and elevation of factor VIII in a child with ischemic stroke: a case report.

A 4-year-old boy was admitted with left hemiplegia. Thrombophilia marker examination resulted with factor V Leiden mutation heterozygosity, a deficiency of antithrombin III and a high level of factor VIII. Cranial computed tomography scan revealed an ischemic infarct in the region of right anterior cerebral artery. In the literature, combinations of multiple thrombophilia risk factors that trigger cerebral ischemic stroke in children have been emphasized. To our knowledge, this is the first child with these combinations of thrombophilia risk factors and ischemic stroke to be reported in the literature.

A premature infant with h-type tracheoesophageal fistula demonstrated by scintigraphic technique.

PURPOSE: Congenital tracheoesophageal fistula (TEF) without esophageal atresia is commonly known as "H" type. This is extremely rare in infants. The rarity of the condition, its nonspecific symptomatology, and limitations in its demonstration by contrast radiology and endoscopy contribute to delays between first presentation and confirmation of the diagnosis. Our aim was to demonstrate a congenital H-type tracheoesophageal fistula by using Tc-99m sulfur colloid scintigraphy. MATERIALS AND METHODS: A case report. RESULTS: A preterm female infant was born by cesarean section at 34 weeks gestation. After many attempts of feeding, she developed apnea, resolving spontaneously, peroral cyanosis, pallor, hypersalivation, and abdominal distension, even when she was being fed by an orogastric tube. With this complex symptomatology, she was suspected to have an H-type TEF. Because of prematurity and recurrent respiratory problems of the infant and the high risk of aspiration of contrast material during a cineradiographic procedure, and also because of the invasive nature of the endoscopic procedure as well as the requirement of general anesthesia, the diagnosis was made by using Tc-99m sulfur colloid scintigraphy and confirmed at operation. CONCLUSION: Demonstrating a congenital H-type tracheoesophageal fistula by using radionuclide imaging is an easily applicable technique.

A new emerging problem in pediatric emergency units: Synthetic cannabinoids.

Synthetic cannabinoids or synthetic marijuana derivatives known as "Bonzai" in Turkey are addictive substances that affect the brain and have serious side effects. In recent years, the use of these substances has also been increasing in our country as well as all over the world. These substances present with various names and brands, the most common names in Turkey are "Bonzai" and "Jamaika". In this study, we aimed to evaluate the demographic and clinical findings of these patients. We retrospectively evaluated patients admitted to our pediatric emergency department between March 2013 and March 2015 due to the complaints developing after the use of "Bonzai" in terms of age, gender, admission date, complaint, clinical findings, laboratory findings, consuming method of the substance, additional substance use, observation durations, hospitalization status and mortality rates. The most common complaint was faintness and drowsiness. Changes in the state of consciousness, tachycardia, hypertension were the most common clinical findings. Most common consuming method of the drug was in the form of smoking. The rate of "Bonzai" use among adolescents is increasing every day. Physicians in emergency departments should be educated on this issue and it must be kept in mind that most of the patients admitted are with complaints related to changes in the cognitive state (of consciousness) but they can also apply with complaints about all other systems.

Bilateral choanal atresia in premature monozygotic twins.

Bilateral congenital choanal atresia is a relatively rare anomaly of the upper airway, which may cause life-threatening respiratory emergency and require rapid diagnosis and treatment. This condition usually occurs sporadically, but has also been rarely described in siblings. We present monozygotic premature twin infants with identical findings of bilateral choanal atresia and no other associated anomalies. To our knowledge, this is the first report of such an occurrence.

Neuron-specific enolase as a marker of the severity and outcome of hypoxic ischemic encephalopathy.

The aim of this study was to evaluate serum concentrations of neuron-specific enolase (NSE) as a marker of the severity of hypoxic ischemic encephalopathy (HIE) and to elucidate the relation among the concentrations of NSE, grade of HIE and short-term outcome. Forty-three asphyxiated full-term newborn infants who developed symptoms and signs of HIE (Group 1) and 29 full-term newborn infants with meconium-stained amniotic fluid but with normal physical examination (Group 2) were studied with serial neurological examination, Denver developmental screening test (DDST), electroencephalogram and computerized cerebral tomography (CT) for neurological follow-up. Thirty healthy infants were selected as the control group. In the patient groups, two blood samples were taken to measure NSE levels, one between 4 and 48 h and the other 5-7 days after birth. Serum NSE levels were significantly higher in infants with HIE compared to those infants in Group 2 and control group. The mean serum concentrations of the second samples decreased in all groups studied but they were significantly higher in Group 1 compared to those in Group 2. Serum NSE concentrations of initial samples were significantly higher in patients with stage III HIE than in those with stages II and I. The sensitivity and specificity values of serum NSE as a predictor of HIE of moderate or severe degree (cut-off value 40.0 microg/l) were 79 and 70%, respectively, and as a predictor of poor outcome (cut-off value 45.4 microg/l) were calculated as 84 and 70%, respectively. The predictive capacity of serum NSE concentrations for poor outcome seems to be better than predicting HIE of moderate or severe degree. However, earlier and/or CSF samples may be required to establish serum NSE as an early marker for the application of neuroprotective strategies.

Wolf-Hirschhorn syndrome with posterior intraorbital coloboma cyst: an unusual case.

Wolf-Hirschhorn syndrome (WHS) is associated with partial deletion of short arm of chromosome 4, and characterized by severe growth retardation. Other characteristic features are microcephaly, intellectual handicap, Greek helmet facies and closure deficits such as cleft lip or cleft palate, coloboma of the eye, and cardiac septal defect. We herein present a new case of WHS with bilateral iris colobomata and a left intraorbital large coloboma cyst causing exophthalmos.

VACTERL-H with triphalangeal thumb and hypothyroidism in a female patient.

The association of vertebral, cardiac, renal, limb anomalies, anal atresia, tracheo-esophageal fistula (VACTERL) with hydrocephalus (VACTERL-H) has recently been described. Both X linked and autosomal recessive forms have been described which are mostly indistinguishable clinically. Here we report a female newborn infant with hydrocephalus detected antenatally, oesophageal atresia with tracheo-oesophageal fistula, anal atresia, renal and vertebral anomalies, and triphalangeal thumb as well as primary hypothyroidism.