Comparative study on effects of volume-controlled ventilation and pressure-limited ventilation for neonatal respiratory distress syndrome / 소아과

PURPOSE: In contrast with traditional time-cycled, pressure-limited ventilation, during volume-controlled ventilation, a nearly constant tidal volume is delivered with reducing volutrauma and the episodes of hypoxemia. The aim of this study was to compare the efficacy of pressure-regulated, volume controlled ventilation (PRVC) to Synchronized intermittent mandatory ventilation (SIMV) in VLBW infants with respiratory distress syndrome (RDS). METHODS: 34 very low birth weight (VLBW) infants who had RDS were randomized to receive either PRVC or SIMV with surfactant administration : PRVC group (n=14) and SIMV group (n=20). We compared peak inspiratory pressure (PIP), duration of mechanical ventilation, and complications associated with ventilation, respectively with medical records. RESULTS: There were no statistical differences in clinical characteristics between the groups. After surfactant administration, PIP was significantly lower during PRVC ventilation for 48hrs and accumulatevive value of decreased PIP was higher during PRVC ventilation for 24hrs (P <0.05). Duration of ventilation and incidence of complications was no significant difference. CONCLUSION: PRVC is the mode in which the smallest level of PIP required to deliver the preset tidal volume in VLBW infants with RDS, adaptively responding to compliance change in lung after surfactant replacement.

The risk factors and prognosis associated with neonatal pulmonary hemorrhage / 소아과

PURPOSE: Although neonatal pulmonary hemorrhage is rare, it is associated with high mortality. We aimed to evaluate the risk factors associated with pulmonary hemorrhage in preterm infants and to describe the clinical course, including neonatal morbidity, of infants who developed pulmonary hemorrhage. METHODS: We performed a retrospective case-control study of 117 newborn infants aged less than 37 gestational weeks admitted to the neonatal intensive care unit of Daegu Fatima Hospital between January 1995 and December 2008. Control group infants without pulmonary hemorrhage were matched according to the gestational age, duration of mechanical ventilation, and birth weight range (< or =100 g). Pulmonary hemorrhage was defined as the presence of hemorrhagic fluid in the trachea and severe respiratory decompensation. RESULTS: Pulmonary hemorrhage occurred in 17 cases of very low birth weight infants (VLBW; birth weight < 1,500 g; median age, 3 days) and 22 cases of low birth weight infants (LBW; 1,500 g < or = birth weight < 2,500 g; median age, 1 day). Antenatal maternal glucocorticoid treatment significantly reduced the incidence of pulmonary hemorrhage in VLBW infants. Low APGAR score (< or =3 at 1 min) and acidosis at birth were associated with significantly high incidence of pulmonary hemorrhage in LBW infants. CONCLUSION: Antecedent factors and timing of pulmonary hemorrhage of LBW infants were different from those of VLBW infants. The mortality rates of VLBW and LBW infants were 88.2% and 45.5%, respectively. Pulmonary hemorrhage was the principal cause of death in 66.6% VLBW infants and 40.0% LBW infants.

The effects of neonatal ventilator care or maternal chorioamnionitis on the development of bronchopulmonary dysplasia / 소아과

PURPOSE: Advances in neonatal intensive care have improved the survival rate of low-birth-weight infants, but mild bronchopulmonary dysplasia (BPD) with the accompanying need for prolonged oxygen supplement remains problematic. Maternal chorioamnionitis and neonatal ventilator care affect the development of BPD. This study aimed to examine whether maternal chorioamnionitis or neonatal ventilator care affect the development of BPD dependently or independently. METHODS: We performed a retrospective study of 158 newborn infants below 36 weeks of gestational age and 1,500 gm birth weight admitted to the neonatal intensive care unit of Daegu Fatima Hospital between January 2000 and December 2006. We analyzed the incidence of BPD according to maternal chorioamnionitis and neonatal ventilator care. Result: Histologic chorioamnionitis was observed in 50 of 158 infants (31.6%). There were no significant differences in the development of BPD (P=0.735) between the chorioamnionitis (+) and chorioamnionitis (-) groups. In the multiple regression analysis, ventilator care (OR=7.409, 95% CI=2.532-21.681) and neonatal sepsis (OR=4.897, 95% CI=1.227-19.539) affected the development of BPD rather than maternal chorioamnionitis (OR=0.461, 95% CI=0.201-1.059). CONCLUSION: Ventilator care or neonatal sepsis may play a role in the development of BPD rather than maternal chorioamnionitis.

The effects of early surfactant treatment and minimal ventilation on prevention of bronchopulmonary dysplasia in respiratory distress syndrome / 소아과

PURPOSE: Early surfactant treatment and minimal ventilation, bronchopulmonary dysplasia needed prolonged oxygen supplement is a problem. This study aimed to report the effects of early surfactant treatment and minimal ventilation on the prevention of bronchopulmonary dysplasia in respiratory distress syndrome. METHODS: We retrospectively studied 139 premature newborn infants (gestational age, 36 weeks; birth weight, 1,500 gm) admitted to the neonatal intensive care unit of Daegu Fatima Hospital between January 2001 and December 2006. We analyzed the occurrence of bronchopulmonary dysplasia with respect to ventilator care and surfactant treatment. RESULTS: The incidence of bronchopulmonary dysplasia was significantly higher with prolonged ventilator care, moderate to severe respiratory distress syndrome, and low Apgar score (P<0.001). Despite early surfactant treatment and minimal ventilation, mild bronchopulmonary dysplasia occurs in a considerable number of patients with mild respiratory distress syndrome. The patient group with low Apgar scores required ventilator care for a prolonged period (P=0.020). CONCLUSION: Early surfactant treatment and minimal ventilation shortens the duration of ventilator care; however, the preventive effe cts on bronchopulmonary dysplasia are limited. The refore, not only early surfactant treatment and minimal ventilation but also appropriate management in the delivery room is essential.

The outcomes of retinopathy of prematurity in relation to duration of low dose oxygen therapy / 소아과

PURPOSE: This study aimed to determine the influence of low-dose oxygen (FiO2 <25%) therapy through nasal cannulae on the progress and prognosis of retinopathy of prematurity (ROP) as well as methods of preventing ROP. METHODS: Our subjects comprised premature infants (gestation period <37 weeks; birth weight <1,750 g) born in Daegu Fatima Hospital between February 1,2001 and January 31,2006. We retrospectively reviewed and analyzed the medical records of 273 patients who were available for eye examination and follow up over 6 months. RESULTS: The factors maximally influencing the occurrence of ROP were low gestation age and low birth weight. We observed that the incidence of ROP increased with the increasing duration of low-dose oxygen therapy. ROP onset was delayed during ongoing oxygen therapy; however, rapid progression of ROP occurred after the discontinuation of oxygen therapy among premature infants up to the prethreshold stage. CONCLUSION: To prevent of occurrence of severe ROP and its rapid progression, the period of low-dose oxygen therapy needs to be shortened. Moreover, frequent eye examinations should be performed after the discontinuation of oxygen therapy.

Study of the risk factors for pulmonary interstitial emphysema related to mechanical ventilator care / 소아과

PURPOSE: Pulmonary interstitial emphysema (PIE) primarily occurs in preterm infants suffering from respiratory distress syndrome (RDS) and kept under mechanical ventilator care. Therefore, this study aimed to examine various risk factors for PIE, to identify conditions that can decrease the possibility of PIE development. METHODS: PIE classification was conducted for 183 patients diagnosed to have RDS and receiving mechanical ventilator care with pulmonary surfactant between March 2000 and February 2007. The characteristics of each patient were analyzed through retrospective examination of their medical histories. RESULTS: Among 183 patients, 17 had PIE; all factors, including birth weight, gestational age, RDS grade III or above, chorioamnionitis, and premature rupture of membranes, were statistically significant (P<0.05). The period of mechanical ventilator use was statistically significant, but the peak mean airway pressure and peak partial pressure of inspired oxygen were not. PIE mainly occurred on the right side or both sides rather than the left side and mostly developed within 72 h. The PIE group showed higher mortality rate than the control group, and the major cause of mortality was pneumothorax. CONCLUSION: Risk factors for PIE in infants suffering from RDS and kept under mechanical ventilator care include low gestational age, low birth weight, chorioamnionitis, and premature rupture of membranes. If any risk factors are noted, the infant must be observed closely for at least 72 h after birth.

Normative data of penile length in Korean newborns / 소아과

PURPOSE: As modern society has became more open, interest in healthy internal and external growth has increased, including that pertaining to penile length in children. A micropenis is defined as one where penile length is more than 2 SD (standard deviation) below the mean, and it can be traced back to chromosome and endocrine disorders. The authors executed this study to suggest guidelines for the study of the micropenis and standard information for penile length in Korean newborns. METHODS: The subjects of this study were 168 male infants between 37 and 42 weeks of gestational age, none of whom had any complications during pregnancy or birth; each had been born in Daegu Fatima Hospital between February and June 2007. Penile length was measured using conventional stretched penile length measurement (CPLM) and syringe methods. RESULTS: Penile length was 3.02+/-0.25 cm (F=36.467, R(2)=0.180, P<0.001) when measured with CPLM, and 3.29+/-0.26 cm (F=9.149, R(2)=0.052, P<0.001) with the syringe method. There was no statistically significant difference in the penile length of newborn infants as a result of taking measurements with the two methods, and both methods showed significance at 0.631 in terms of Pearson's correlation coefficient, at the level of P=0.01. CONCLUSION: In this study, penile length tended to be longer when gestational age was longer, and a micropenis can be assumed to be one less than 2.5 cm using CPLM and less than 2.8 cm using the syringe method. In the case of a concealed penis, the syringe method is helpful. When a micropenis is assumed, close observation by outpatient department personnel, and additional endocrine and chromosome studies should be undertaken after sufficiently consulting the parents.

Normative data of penile length in Korean newborns / 소아과

PURPOSE: As modern society has became more open, interest in healthy internal and external growth has increased, including that pertaining to penile length in children. A micropenis is defined as one where penile length is more than 2 SD (standard deviation) below the mean, and it can be traced back to chromosome and endocrine disorders. The authors executed this study to suggest guidelines for the study of the micropenis and standard information for penile length in Korean newborns. METHODS: The subjects of this study were 168 male infants between 37 and 42 weeks of gestational age, none of whom had any complications during pregnancy or birth; each had been born in Daegu Fatima Hospital between February and June 2007. Penile length was measured using conventional stretched penile length measurement (CPLM) and syringe methods. RESULTS: Penile length was 3.02+/-0.25 cm (F=36.467, R(2)=0.180, P<0.001) when measured with CPLM, and 3.29+/-0.26 cm (F=9.149, R(2)=0.052, P<0.001) with the syringe method. There was no statistically significant difference in the penile length of newborn infants as a result of taking measurements with the two methods, and both methods showed significance at 0.631 in terms of Pearson's correlation coefficient, at the level of P=0.01. CONCLUSION: In this study, penile length tended to be longer when gestational age was longer, and a micropenis can be assumed to be one less than 2.5 cm using CPLM and less than 2.8 cm using the syringe method. In the case of a concealed penis, the syringe method is helpful. When a micropenis is assumed, close observation by outpatient department personnel, and additional endocrine and chromosome studies should be undertaken after sufficiently consulting the parents.

A Case of Cornelia de Lange Syndrome with Imperforate Anus

Cornelia de Lange syndrome is characterized by severe growth and mental retardation, characteristic face, and a low-pitched, weak, growling cry, which was first described by Cornelia de Lange in 1933. We have recognized Cornelia de Lange syndrome with imperforate anus in a male neonate. The patient had the typical facial appearance: micrognathia, confluent eyebrows, long curly eyelashes, underdeveloped orbital arches, long philtrum, thin lip, downturned angles of the mouth, anteverted nares, low-set ears, high-arched palate, and generalized hirsutism. His karyotype was normal, but skeletal abnormalities of the hands, cryptorchism, and imperforate anus with rectourethral fistula. A colostomy was performed on the third day of hospitalization.

Usefulness of auditory brainstem response as early predictor of kernicterus in early breast-feeding jaundice / 소아과

PURPOSE: The present study examined the etiology and risk factors of the early breast-feeding jaundice and the usefulness of auditory brainstem response test as early predictor of kernicterus. METHODS: Medical records of neonatal jaundice in newborn admitted to Daegu Fatima Hospital between September 2005 and May 2006 were analyzed prospectively. Infants were grouped according to feeding method:breast feeding group (breast feeding only, n=23), mixed feeding group (breast feeding mainly plus addition of fomula feeding, n=13). RESULTS: There were no significant differences in gestational age, birth weight, sex, duration of phototherapy, serum bilirubin and hemolytic evidence between the two study groups. First visiting day of life at out patient department was significantly delayed in breast feeding group (8.7+/-3.6 day) compared to mixed feeding group (6.0+/-1.9) (P=0.009). Weight loss was significantly severe in breast feeding group compared to mixed feeding group (P<0.05). In auditory brainstem response test, loss of Wave V in 3 cases was observed and recoverd after blood exchange transfusion in follow up test. Wave III latency had significant correlation to serum bilirubin in auditory brainstem response test (70 dB) (P=0.002). CONCLUSION: Our study suggest that further education about breast feeding and follow up within the first postnatal week would be necessary for early detection and prevention of early breast-feeding jaundice. Test of serum bilirubin and auditory brainstem response would be helpful in determination of blood exchange transfusion.

Changes of neurodevelopmental outcomes and risk factors of very low birth weight infants below 1,500 g, in the last 10 years / 소아과

PURPOSE: As a result of advances in neonatal intensive care and perinatal care, neurodevelopmental outcomes of very low birth weight infant(VLBWIS) is expected to lead to improvement. The aim of this study was to report neurodevelopmental outcomes and risk factors of neurologic impairment of very low birth weight infants during the past 10 years. METHOD: We performed a retrospective study of 447 newborn infants below 1,500 gm admitted to neonatal intensive care unit of Taegu Fatima Hospital between Janury 1996 and December 2004. Infants were subdivided into group 1(Jan. 1996 to Dec. 1998), group 2(Jan. 1999 to Dec. 2001), and group3(Jan. 2002 to Dec. 2004). We analyzed epidemiologic data to study changes of neurodevelopmental outcomes and risk factors of neurologic impairment. RESULT: The incidence of cerebral palsy and developmental delay decreased significantly in periods 2 and 3(vs period 1; cerebral palsy 10 percent, developmental delay; 18 percent, P<0.05). Periventricular leukomalacia incidence decreased in period 3(vs period 1; 14.5 percent, P<0.05). The overall survival rate of VLBWIS increased significantly in period 2 and 3(vs period 1; 90.0 percent, P<0.05). The risk factors of neurologic impairment are long-term ventilator care(above 1 wk), low Apgar score, low gestational age and low birth weight. CONCLUSION: In the most recent 10 years, neurologic impairments of VLBWIS significantly decreased, as a result of advances in neonatal intensive care and perinatal care.

A Case of Recurrent Bacterial Meningitis Associated with Lumbosacral Dermal Sinus during Early Infancy / 대한소아신경학회지

Acute bacterial meningitis is an uncommon, potentially life-threatening infection and recurrent episodes of bacterial meningitis are rarely seen. But when they occur, an exhaustive search for the mechanisms underlying the recurrent episodes must be pursued, especially some possible routes of migration of bacteria to the cerebrospinal fluid. Dermal sinus tracts are common skin manifestations seen with spinal dysraphism and may be associated with recurrent meningitis. Here, we present a case of a 2-month-old girl who had a small dimple on the lower lumbosacral area. She was attacked twice by purulent bacterial meningitis due to a dermal sinus tract and a tethered cord. She was treated with the excision of the tract, the removal of the dermoid and the detethering of the cord. Staphylococcus and Klebsiella were cultured separately and assumed to be causative agents. The lesion was suspected on the physical examination and demonstrated by lumbosacral magnetic resonance imaging.

A Study on the Effects of Early Surfactant Replacement and Gentle Ventilation in the Treatment of Respiratory Distress Syndrome / 소아과

PURPOSE: Recently, early surfactant replacement and tidal volume based gentle ventilation has been a fundamental treatment of respiratory distress syndrome (RDS). The aims of this study were to survey the changes in ventilator care duration and rate of complication in RDS groups. METHODS: We performed a retrospective study of 255 newborn infants less than 1, 500 g admitted to the neonatal intensive care unit (NICU) and discharged from January 1999 to December 2003. 141 of 255 newborn infants were RDS groups that required invasive management, such as endotracheal intubation, surfactant replacement and assisted ventilation. We analyzed epidemiologic data to study the changes in ventilator care duration and outcome of RDS groups. RESULTS: Of 141 RDS groups, 135 were mild to moderate RDS groups and only 6 were severe RDS groups. 24 (17.8%) of 135 mild to moderate RDS groups and 3 (50%) of 6 severe RDS groups were antenatal no use of maternal dexamethasone. 127 (90.1%) of 141 RDS groups underwent replacement of surfactant during 3 hours after birth. 121 (85.9%) weaned within 48 hours. CONCLUSION: Our study shows a decreased frequency of severe RDS by a antenatal use of maternal dexamethasone and decreased duration of ventilator care by early surfactant replacement and gentle ventilation.

Development of Periventricular Leukomalacia and Severe Intraventricular Hemorrhage in Very Low Birth Weight Newborns and Relationship with Ventilator Care (Study of Ventilator Care as a Risk Factor of PVL and PV-IVH) / 소아과

PURPOSE: It has been suggested that changes in cerebral blood flow by ventilator care could be a risk factor in periventricular leukomalacia (PVL) and severe periventricular-intraventricular hemorrhage (PV-IVH). The study aims to assess the relationship between perinatal clinical events, including ventilator care, and the development of PVL and severe PV-IVH; especially, whether ventilator care could be causers of PVL and severe PV-IVH as an individual risk factor. METHODS: Among 255 very low birth weight infants who survived in the Fatima neonatal intensive care unit from January 1999 to December 2003, 15 infants with PVL and eight infants with severe PV-IVH were classified as a study group, while 231 infants were enrolled as a control group. The analysis was performed retrospectively with medical records. RESULTS: Twenty four infants were diagnosed with PVL or severe PV-IVH. Asphyxia, recurrent apnea, sepsis, acidosis and ventilator care were significantly increased in the PVL goup. Asphyxia, recurrent apnea, RDS, acidosis and ventilator care were significantly increased in the severe PV-IVH group. CONCLUSION: Infants with PVL or severe PV-IVH may have multiple perinatal risk factors including asphyxia, recurrent apnea, sepsis, acidosis, RDS and ventilator care. Because most patients with ventilator care have multiple perinatal risk factors, ventilator care does not cause PVL and severe PV- IVH independently. Therefore, incidences of PVL and severe PV-IVH can be decreased by not only gentle ventilation, but also more professional antenatal care.

High Frequency Oscillatory Ventilation with Inhaled Nitric Oxide Combined Therapy in The Intractable Respiratory Failure Neonates / 소아과

PURPOSE: Inhaled nitric oxide(iNO) has been known to improve oxgenation in newborns with persistent pulmonary hypertension(PPHN), but it's not clear that iNo treatment is effective in cases of acute, hypoxic respiratory failure and premature infants. In our study, high frequency oscillatory ventilation(HFV) with iNO combined therapy was tried in intractable respiratory failure neonates who showed no response to conventional ventilator therapy. METHODS: Between May 2000 and December 2002, 18 newborns with intractable respiratory failure were treated with HFV with iNO combined therapy. According to the respective response times, the newborns were divided into rapid, intermediate, and non-response groups. Clinical response was defined as when the oxygenation index(OI) was reduced more than 20%. RESULTS: Six neonates(33.3%) showed a rapid response, eight(44.4%) an intermediate response, and four(22.2%) no response. Four had PPHN(22.2%), Three meconium aspiration syndrome(16.7%), three pneumonia(33.3%), and two sepsis(11.1%). Six neonates(33.3%) died, five from respiratory distress syndrome(RDS) and one from sepsis. Of them, one was a full-term neonate and five were premature infants under 35 weeks. Their complications were pneumothorax, pulmonary hemorrhage, pulmonary edema, and congestive heat failure. CONCLUSION: HFV with iNo combined therapy is not effective in intractable respiratory failure, lower gestational age, RDS, and sepsis. However, iNO may be beneficial to patients with acute, hypoxic respiratory failure.

A Study on the Effects of the Early Use of Nasal CPAP in the Weaning of Mechanical Ventilators

PURPOSE: This study was conducted for the use of nasal continuous positive airway pressure (CPAP), by comparing the early use of non-invasive nasal CPAP with low intermittent mandatory ventilation(low IMV) and endotracheal CPAP in weaning a mechanical ventilator from infants with moderate respiratory distress syndrome(RDS). METHODS: Thirty infants in the study group, with moderate RDS from November 2001 to June 2002, were administered surfactants and treated with the mechanical ventilator, and applied the nasal CPAP in weaning. Thirty infants of the control group, from January 1999 to September 2001, were applied low IMV and endoctracheal CPAP in weaning. RESULTS: There were no significant differences in the characteristics, the severity of clinical symptoms, the initial laboratory findings and settings of the mechanical ventilator. After weaning, the study group showed no significant changes in PaCO2. However, the control group showed a slight CO2 retension after one and 12 hours. Twenty eight infants(93.3%) of the study group and 24 infants(80%) of the control group were successfully extubated. The primary cause of failure was apnea. There were no significant differences in the duration of weaning and the mechanical ventilator treatment between the groups. Complications in weaning were related to the fixation of nasal CPAP and the mechanical problems caused by endotracheal tube. CONCLUSIONS: Aggressive weaning is possible for moderate RDS, in which the nasal CPAP was used without the low IMV and the endotracheal CPAP process. It had no difficulties. In conclusion, the nasal CPAP is an adequate weaning method for moderate RDS.

A Study on the Changes in Ventilator Care Rate and Outcome of Very Low Birth Weight Infants During Last Four Years

PURPOSE: Recently there has been a decrease in ventilator care rate and duration of very low birth weight infants(VLBWI) in Fatima Hospital. The aims of this study were to survey the frequency and duration of ventilation in VLBWI and to develop a non-invasive neonatal intensive care unit (NICU) policy. METHODS: We performed a retrospective study of 284 newborn of infants less than 1,500 gm admitted to NICU and discharged from January 1998 to December 2001. Patients were intubated or applied continuous positive airway pressure(CPAP) via nasal prong immediately after presenting signs of respiratory distress. We analyzed epidemiologic data to study the changes in ventilator care rate, duration and outcome of ventilator care groups. RESULTS: Of 284 newborn infants, 146 required invasive management, such as endotracheal intubation and assisted ventilation. The characteristics, the severity of clinical symptoms and laboratory findings in ventilator care groups at birth showed no significant differences. The annual proportion of infants requiring assisted ventilation decreased according to increasing gestational age. The median duration of ventilation decreased markedly from 6.0 days in 1998 to 2.7 days in 2001. Final complications and outcomes in ventilator care groups showed no significant differences. CONCLUSION: Our study shows a significant reduction in the invasiveness of the treatment of VLBW infants, which was not associated with an increased mortality or morbidity. A non-invasive strategy for the VLBW infant with minimal to moderate respiratory distress after birth in NICU is better than immediate invasive management. Non-invasive nasal CPAP is a simpler and safer method than invasive assisted ventilation.

Effect of Nasal Continuous Positive Airway Pressure after Early Surfactant Therapy in Moderate Respiratory Distress Syndrome

PURPOSE: Early surfactant therapy with either gentle ventilation, high-frequency ventilation or aggressive weaning of mechanical ventilation are principles for the treatment of respiratory distress syndrome(RDS). We studied to determine the accessibility of noninvasive nasal continuous positive airway pressure(CPAP) rather than mechanical ventilation by invasive intubation after early surfactant therapy. METHODS: The study group consisted of 14 infants who were born and diagnosed with moderate respiratory distress syndrome and received early surfactant therapy with nasal CPAP of PEEP 5-6 cm H2O within two hours after birth in the Fatima neonatal intensive care unit for two years from January 1999 to August 2001. The control group consisted of 15 infants who were diagnosed with the disease and could be weaned from mechanical ventilator within five days after birth during the same period. RESULTS: The characteristics, the severity of clinical symptoms and laboratory findings in the two groups at birth showed no significant difference. Neither did the interim analysis of laboratory data in two groups. Of 14 infants in the study group who received nasal CPAP after early surfactant therapy, only two infants showed weaning failure with this therapy. In the response cases, duration of CPAP was five days and mean airway pressure was 5.4+/-0.5 cm H2O. Two had the complication of CPAP with abdominal distension. Final complications and outcomes in the two groups showed no signifcant difference(p>0.05). CONCLUSION: The clinical courses in the two groups showed no significant difference. Therefore, we suggest that early surfactant therapy with noninvasive nasal CPAP is a simple and safe method rather than aggressive weaning after invasive mechanical ventilation in moderate respiratory distress syndrome.

A Case of Incontinentia Pigmenti with Developmental Brain Malformation

Incontinentia pigmenti is a rare neurocutaneous syndrome characterized by vesiculobullous skin disease in neonates and infants, a noninfectious disease that should be distinguished from infectious diseases with the neonatal seizure or encephalopathy. This disease is X-linked dominant with Xq28 region abnormalities and often associated with developmental defects of the ocular, skeletal, dental, and central nervous system. Central nervous system involvement in the neonatal period, or complicated by encephalopathy, may cause severe neurologic impairment, retardation or even death. We experienced a case of incontinentia pigmenti in a three-day-old female patient who had characteristic papulovesicular skin lesions and partial seizures with secondary generalization. Histopathological examination favored the diagnosis of incontinentia pigmenti and a brain MRI showed undifferentiated white matters with periventricular nodular lesions.

A Study on the Risk Factors of Periventricular Leukomalacia in Combined Use with Gentle Ventilation and High-Frequency Ventilation

PURPOSE: It is suggested that persistent hypocarbia caused by ventilator therapy could be a risk factor in PVL. The study is aimed to discover whether for preventing hypocarbia with combined use of gentle ventilation and high frequency ventilation, other factors would be causers of PVL. METHODS: Among 45 infants who were born and survived through ventilator treatment in the Fatima neonatal intensive care unit for four years from April 1996 to June 1999, 15 infants with PVL were classified as a study group and 30 without PVL as control group. The analysis was performed retrospectively with medical records. Ventilator treatment was based on the combined use of ventilation by means of the flow interruptor type of Infant Star . The aggressive weaning was performed when the clinical state, chest X-ray and arterial blood gas analysis became stabilized. RESULTS: Among 15 cases with PVL : 9 cases(60.0%) with fetal distress, 1 case(6.6%) with placenta previa, 1 case(6.6%) with placenta abruptio. In the relationship between PaCO2 variance on arterial blood gas analysis and PVL, the highest average of PaCO2 is 44.9 +/- 7.8 mmHg in the study group and 45.0 +/- 10.5 mmHg in the control group, which means there was not statistically significant difference. The PaCO2 concentration lower than 25 mmHg for three days appeared in one case in the study group. CONCLUSIONS: In cases of preventing hypocarbia by combined use of ventilation, it is suggested that the birth history and weaning method is important as risk factor of PVL.