Holoprosencephaly is a developmental malformation complex of forebrain and midface which arises from incomplete cleavage of the embryonic forebrain. It is subdivided into alobar, semilobar and lobar types based on the degree of growth disturbance within the anterior wall of the telencephalon, particularly in the midline. Cyclopia is the most severe form of alobar holoprosencephaly presenting a single median eye and a blind-ending proboscis usually located above the eye. We report a case of alobar holoprosencephaly with cyclopia and proboscis in premature infant.
Holoprosencephaly* ; Humans ; Infant, Newborn ; Infant, Premature ; Prosencephalon ; Telencephalon

Enterobacter cloacae, a gram negative bacillus is an increasingly frequent cause of nosocomial pediatric infection. It can cause infection of postsurgical wounds, meningitis, and infection of the gastrointestinal, urinary, and respiratory tracts. Newborn infants often are colonized by Enterobacter species in the gastrointestina tract or respiratory tract soon after birth, and a acquisition of hospital strains in immunocompromised newborn infants is common. Neonatal pneumonia has been associated with many infectious agents. Pneumatocele, a cyst-like lesion that develops within the lung parenchyma, is an unusual complication of pneumonia in the neonate, especially in the preterm neonate. Pneumatocele has been reported to occur with Staphylococcus aureus, Escherichia coli, Klebsiella pneumoniae, Streptococcus pneumoniae, and Pseudomonas aeruginosa infections. We describe 2 cases of premature neonates with pneumonia and subsequent pneumatocele formation caused by Enterobacter cloacae.
Bacillus ; Colon ; Enterobacter cloacae* ; Enterobacter* ; Escherichia coli ; Humans ; Infant, Newborn* ; Klebsiella pneumoniae ; Lung ; Meningitis ; Parturition ; Pneumonia ; Pseudomonas aeruginosa ; Respiratory System ; Staphylococcus aureus ; Streptococcus pneumoniae ; Wounds and Injuries

Congenital cytomegalovirus (CMV) infection is one of the most frequent congenital infections in neonates. It can manifest as asymptomatic infection in 90% or symptoms may appear in 10% of the patients. Asymptomatic congenital CMV infection is likely to be a leading cause of sensorineural hearing loss, mental retardation and microcephaly. Incontinentia pigmenti is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eyes, CNS, teeth, hair, and nail. It is regarded as an X-linked dominent genetic disorder. We are reporting a case of neonate who presented with delayed development and bilateral hearing loss due congenital CMV infection which was incidentally assoicated with incontinentia pigmenti.
Asymptomatic Infections ; Cytomegalovirus ; Ectoderm ; Hair ; Hearing Loss ; Hearing Loss, Bilateral ; Hearing Loss, Sensorineural ; Humans ; Incontinentia Pigmenti* ; Infant, Newborn ; Intellectual Disability ; Microcephaly ; Tooth

Joubert syndrome is a rare autosomal recessive disorder characterized by cerebellar vermian hypoplasia, hypotonia, developmental delay, abnormal respiratory patterns and abnormal eye movements. It is usually diagnosed during infancy or thereafter by hypotonia and developmental delay. We experienced a case of Joubert syndrome presenting in newborn period manifesting as unexplainable episodic hyperpnea and apnea.
Apnea ; Eye Movements ; Humans ; Infant, Newborn ; Muscle Hypotonia

An H-type tracheoesophageal fistula is a rare congenital condition of which incidence is approximately 1: 50, 000-80, 000 births. Although patient usually has recurrent respiratory symptoms such as choking episodes and cyanotic spells, its rarity and atypical symptoms may delay its detection. Besides, conventional esophagogram often fail to find the defect. We reported a case of H-type tracheoesophageal fistula, diagnosed by tube esophagram with infant in the prone position. During the test, contrast medium was injected while the catheter is slowly withdraws from lower esophagus.
Airway Obstruction ; Catheters ; Esophagus ; Humans ; Incidence ; Infant ; Parturition ; Prone Position* ; Tracheoesophageal Fistula*

PURPOSE: This study was conducted to evaluate the comparability and accuracy of body temperatures measured at three different sites: rectum, axilla and between skin and mattress in the newborn infants. METHODS: 168 term and preterm infants admitted to NICU at Kyungpook University Hospital were enrolled in the study. Glass and digital thermometers were used for the simultaneous measurement of rectal, axilla and skin-to-mattress temperatures. RESULTS: Among three different body temperatures measured by glass thermometer, the rectal temperatures recorded the highest in the bassinet, while no differences were found in the incubator. Rectal temperature by glass thermometer agreed most closely with axillary temperature by digital thermometer. The digital thermometers took significantly less time to measure temperatures from rectum and axilla than glass thermometer. Phototherapy did not influence body temperature measured in the incubator. CONCLUSION: In the bassinet, axilla temperature measured by a digital thermometer was as reliable as rectal temperature measured by a glass thermometer. Skin-to-mattress temperature provides an accurate, safe and easily accessible alternative site while infants are in the incubator.
Axilla ; Body Temperature ; Glass ; Gyeongsangbuk-do ; Humans ; Incubators ; Infant ; Infant, Newborn* ; Infant, Premature ; Phototherapy ; Rectum ; Skin ; Thermometers

PURPOSE: Cholestatic liver disease is a frequent complication of prolonged parenteral nutrition, especially in premature infants. The purpose of present study is to evaluate the clinical and biochemical efficacy of ursodeoxycholic acid (UDCA) in premature infants with total parenteral nutrition (TPN) cholestasis. METHODS: Retrospective chart review of 31 prematures with TPN cholestasis in Neonatal Intensive Care Unit of Chonnam University Hospital from January 1995 and December 2002 was done. Prematures were divided into two groups based on UDCA treatment: the study group (n=22, with UDCA treatment) and the control group (n=9, without UDCA treatment). Treatment efficacy of UDCA was evaluated by monitoring the changes of serum direct bilirubin level for more than one to two months. RESULTS: In study group, cholestasis appeared at a mean age of 23+/-18.8 days after a mean of 21+/-19.7 days of TPN. UDCA was initiated (ranged 15-30 mg/kg/day) at a mean age of 54+/-22.3 days for a mean of 32+/-19.0 days. There was no significant difference in decrease of direct bilirubin level between the study and control group. However, the day of initial enteral feeding and full enteral feeding were earlier in control group compared with study group. CONCLUSIONS: This study shows that UDCA treatment is not effective in the treatment of TPN cholestasis among premature infants unlike that of results reported in children and adults. To reduce the incidence of TPN cholestasis in premature infants, early introduction of enteral feeding as soon as possible is most important.
Adult ; Bilirubin ; Child ; Cholestasis* ; Enteral Nutrition ; Humans ; Incidence ; Infant, Newborn ; Infant, Premature* ; Intensive Care, Neonatal ; Jeollanam-do ; Liver Diseases ; Parenteral Nutrition ; Parenteral Nutrition, Total* ; Retrospective Studies ; Treatment Outcome ; Ursodeoxycholic Acid*

PURPOSE: Aortic thrombus is a rare but serious complication in neonates, usually associated with central vessel catheterization. Currently treatment of asymptomatic aortic thrombus in preterm infants is controversial. We evaluated effects of urokinase in preterm infants with aortic thrombi. METHODS: We studied 12 preterm infants in whom umbilical arterial catheterizations were performed and subsequently aortic thrombi were detected. In six patients bolus doses of urokinase 4, 400 IU/kg were injected, followed by continuous infusions of 4, 400 IU/kg/hr. The mean duration of urokinase use was 11 days (6-13 days). The other six patients who did not receive urokinase served as controls. The two groups were compared for changes in the size of thrombi. RESULTS: The initial sizes of aortic thrombi upon detection were similar, although the diagnoses were made earlier in urokinase group than in controls. The days to 50% reduction in size of thrombi were significantly shorter in urokinase group, as were the days to complete resolution. One infant in urokinase group and 2 infants in control group had persistent aortic thrombi up to 90 days of follow-up. Intracranial hemorrhage and disseminated intravascular coagulopathy were absent in all 12 cases. CONCLUSION: Urokinase administration could be an effective therapy for preterm neonates with aortic thrombi. It significantly reduces the size of the thrombus and shortens the days to complete resolution.
Catheterization ; Catheters ; Diagnosis ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Intracranial Hemorrhages ; Thrombosis* ; Urokinase-Type Plasminogen Activator*

PURPOSE: Thymic size can be affected by glucocorticoids which promotes surfactant synthesis in preterm infants. We assessed the hypothesis that the fetal lung maturity in preterm infant correlates with the size of the thymus detected at birth on the routine chest radiograph. We also searched for a possible relationships between thymus size, respiratory distress syndrome (RDS), and the cord blood cortisol level. METHODS: The cardiothymic/thoracic ratios (CT/T) within 6 hours after birth and cord blood cortisol levels were measured in 42 preterm infants admitted to the neonatal intensive care unit of Seoul National University Children's Hospital from June 2002 to December 2003. Multiple linear regression analyses were done to assess the relationships between the CT/T, the perinatal events and the cord blood cortisol levels. The receiver operation characteristic curve analysis was done to evaluate the CT/T in the prediction of RDS. RESULTS: 8 infants (19.0%) developed RDS. The CT/T positively correlated with the birth weight, but not with the gestational age. The CT/T were significantly larger and the cord blood cortisol levels were significantly lower in the preterm infants with RDS than in those without RDS. The cord blood cortisol levels were negatively correlated with the CT/T. The birth weight and RDS were all independently associated with the CT/T. The CT/T less than 0.37 identified infants with RDS with 87.5% sensitivity and 61.8% specificity. CONCLUSION: The larger thymus at birth can be used to identify RDS. The lower cord blood cortisol level may be associated with the larger thymus in RDS.
Birth Weight ; Fetal Blood* ; Gestational Age ; Glucocorticoids ; Humans ; Hydrocortisone* ; Infant ; Infant, Newborn ; Infant, Premature* ; Intensive Care, Neonatal ; Linear Models ; Lung ; Parturition ; Radiography, Thoracic ; Sensitivity and Specificity ; Seoul ; Thymus Gland*

PURPOSE: Recent studies show that chorioamnionitis has an important role in the pathogenesis of bronchopulmonary dysplasia(BPD) and it induces thymic involution. The purpose of this study is to test the usefulness of thymic size at birth as a predictor of BPD. METHODS: This study was conducted on 91 very low birth weight infants of <1, 500 g with mean gestational age of 29.3 weeks and mean birth weight of 1, 161 g who were admitted at NICU of Dong Kang General Hospital for past 4 years of whom 21 infants had BPD. Thymic size was measured on routine chest radiographs taken in the first 3 hours after birth and measured as the ratio between the width of the cardiothymic shadow at the level of the carina and that of the thorax at the costophrenic angles (CT/ T). RESULTS: Correlation of thymic size with gestational age was statistically significant (P=0.003). CT/T of BPD group was smaller than that of non-BPD group (0.27+/-0.06, 0.33+/-0.07, respectively, P<0.01). A significant positive correlation between small thymus at birth and BPD was detected (P=0.003, odds ratio, 21.7), but not in other disease groups. CONCLUSIONS: We concluded that a small thymus at birth on the chest radiograph could be used as an early predictive parameter of the BPD.
Birth Weight ; Bronchopulmonary Dysplasia* ; Chorioamnionitis ; Female ; Gestational Age ; Hospitals, General ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Odds Ratio ; Parturition* ; Pregnancy ; Radiography, Thoracic ; Thorax ; Thymus Gland