• Journal of Chest Surgery
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• v.30 no.7
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• pp.641-646
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• 1997

Understanding of the surgical anatomy of patients with double outlet right ventricle (DORV) is important in the planning of biventricular repair From May 1995 to September 1996, 7 patients underwent biventricular repair for DORV with remote ventricular septal defect. There were 5 males and 2 (tamales. Age at operation varied from 2 to 9 years(mean 3.4$\pm$ 2.7years). Preoperative diagnostic assessment was made by two-dimensional echocardiography and cardiac catheterization. Ventricular septal defect was perimembranous inlet type in all patients. Associated cardiac anomalies were pulmonary atresia in two, pulmonary stenosis in five and tricuspid chordal attachment to zonal septum in five. The operations were performed intraventricular repair and pulmonary enlargement in two, REV operation in two, and Rastelli operation in three. There was no early postoperative deaths and complications. The follow-up period war from 1 month to 18mon1hs, averaging 10: 6.1 months. In the past, we considered the Fontal operation indicative as primary choice when DORV was associated with abnormal tricuspid chordal attachment to the zonal septum, but now we believe that biventricular repair is feasible for those cases by making conal flap or reattachment method. Biventricular repair has theoretic advantages because it estabilishes normal anatomy and physiology, and it was concluded that the precise preoperative evaluation using both echocardiography and cardiac catheterization was essential to the successful surgery.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1080-1084
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• 2003

Purpose : To compare perinatal characteristics, clinical courses, and overall morbidity between respiratory distress syndrome(RDS) with patent ductus arteriosus(PDA) and RDS without PDA in neonates. Methods : Eighty-three neonates who were diagnosed and treated for RDS in the neonatal intensive care unit(NICU) from Jan. 2000 to Dec. 2002 were included in this study. RDS was complicated with PDA(group A) in 17 patients and not complicated in 66(group B). PDA was diagnosed by echocardiogram in neonates with congestive heart failure symptom, cardiac murmur or chest X-ray findings of cardiomegaly or pulmonary edema. A retrospective study was undertaken of the perinatal characteristics and overall morbidity in group A and group B. Results : The birth weight and gestational periods of group A were less compared with group B. There was more perinatal asphyxia in group A. Incidence of overall morbidity such as bronchopulmonary dysplasia, intraventricular hemorrhage and death was higher in group A. Intravenous indomethacin was administered in 17 PDA infants. Conclusion : The perinatal characteristics in the two groups showed a significant difference. Incidence of overall morbidity in the two groups showed significant differences, however, there is no simple conclusion to draw because we didn't do multifactorial analyses to rule out other many risk factors affecting morbidity, such as gestational weeks or birth weight.

• Investigative Magnetic Resonance Imaging
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• v.12 no.2
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• pp.170-177
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• 2008

Purpose : To evaluate usefulness of MR imaging after serial brain US in the high-risk neonates before discharge of the neonatal intensive care unit. Materials and Methods : Retrospective comparison of 412 US and 121 MR scans in 121 neonates and young infants were performed. Grading of germinal matrix/intraventricular hemorrhage (GMH/IVH) was performed and presence of intracranial hemorrhage other than GMH/IVH and parencyma lesions was also analyzed. Results : Among the 242 lateral ventricles, Seven GMH and 46 IVH were additionally detected by MRI. On the other hand, 30 GMH were only detected by US. US demonstrated Grade 1/2/3/4 GMH/IVH in 24/8/13/0 ventricles each, while each grades were identified in 3, 49, 10, 2 ventricles on MR images. Other intracranial lesions additionally detected on MR images were cerebral hemorrhage (n=4), cerebellar hemorrhage (n=4), extraaxial hemorrhage (n=8), diffuse excessive signal change of the white matter (n=72), non-cavitary lesion (n=4), encephalomalacia (n=2), and ventriculomegaly (n=5). Conclusion : MR imaging could be an excellent complimentary study after serial brain US for additional detection of the intracranial pathology, particularly IVH and white matter lesions, though US would be better in follow-up of GMH in some neonates.

• Neonatal Medicine
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• v.17 no.2
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• pp.207-216
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• 2010

Purpose: The aim of this study was to identify the effects of neonatal developmental intervention program (NDT) in promoting motor development and growth and to determine the usefulness of Hammersmith Neonatal Neurological Examination (HNNE) and Neonatal Behavioral Assessment Scale (NBAS) in premature infants. Methods: We performed NDT on selected premature infants (PI, n=42) and compared them with the full term control group (FC, n=20). NDT protocol and development assessment (HNNE, NBAS) were manipulated by the physical therapist in the neonatal intensive care unit. The data of this study were collected prospectively. Results: The PI with GA <34 weeks (VPI) subgroup showed a more use of mechanical ventilator and surfactant, severe bronchopulmonary dysplasia and intraventricular hemorrhage, and patent ductus arteriosus treated surgically than the PI with GA $\geq$34 weeks but less than 37 weeks (LPI) subgroup. The average scores improved significantly in the PI group between the 1st, 2nd, and 3rd assessment by repeated measure (P=0.000). Also, the PI group showed significantly higher total scores and average score at 40 weeks postmenstrual age, P=0.000, respectively than in the FC group. The LPI subgroup showed more weight gain and change in the head circumference between the 1st and 3rd assessment by repeated measure, respectively, P<0.05. The PI group showed no apnea, bradycardia and late sepsis associated with intervention and assessment. Conclusion: The NDT might be a safe and useful intervention to promote motor and growth outcomes in premature infants. Also, the HNNE and NBAS might be safe and useful tools for assessing neurodevelopment in premature infants.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.3
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• pp.1565-1570
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• 2016

Background: Central neurocytomas are rare neuronal neoplasms with a favorable prognosis. They are typically located in the lateral ventricles of the brain and mostly histologically correspond to WHO grade II with a Mib 1 labelling index of <2%. Similar tumors located in the cerebral hemispheres and spinal cord, for example, are called "extraventricular neurocytomas". A few tumors histologically show atypia, mitoses, vascular proliferation and/or necrosis and a Mib 1 index >2 % and are designated as "atypical neurocytomas. Aim: The aim of our study was to describe the common as well as unusual morphologic features and the role of various immunohistochemical stains in the diagnosis of these rare tumors. Materials and Methods: We retrieved and reviewed 35 cases diagnosed between 2001 and 2015. Results: Sixty percent of patients were males, and the mean age was 26 years. 31 cases (88.6%) were intraventricular and 4(11.4%) were extraventricular. Histologically, 6 cases (17.1%) were compatible with "atypical neurocytomas". All cases showed the classic morphology comprising nests and sheets of uniform, round cells with uniform round to oval nuclei with finely speckled chromatin and perinuclear cytoplasmic clearing (halos). All cases also showed delicate, fibrillary, neuropil-like matrices. Other common histologic features included capillary-sized blood vessels in a branching pattern in 57.1%, foci of calcification in 34.3% and perivascular pseudorosettes in 20%. Rare findings included Homer-Wright or true rosettes in 8.6% and ganglioid cells in 2.9%. Synaptophysin was the most consistent and valuable marker, being positive in almost all cases. GFAP positivity in tumor cells was seen in 25.7% of cases. Follow up was available in 13 patients. Of these 9 had histologically typical and 4 had atypical tumors. Only 1 (with an atypical neurocytoma) died, probably due to complications of surgery within one month, while 12 (including 3 with atypical neurocytomas) remained alive. Recurrence developed in 1 of these 12 patients (histologically consistent with typical morphology) almost 9 years after surgery. Only 4 patients, including 2 with atypical tumors, received postoperative radiotherapy, all with surgery in 2010 or later. Overall, prognosis was excellent with prolonged, recurrence free survival and most patients, even without receiving radiation therapy, were alive and well for many years, even a decade or more after surgery, without developing any recurrence, indicating the benign nature of these neoplasms.

• The Journal of the Korean life insurance medical association
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• v.29 no.2
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• pp.29-32
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• 2010

Moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it. MMD is one of cerebrovacular accident,which is treated with sugical maeuver in pediatic neurosurgery. Moyamoya (ie, Japanese for "puff of smoke") characterizes the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. The exact etiology of moyamoya disease is unknown. Some genetic predisposition is apparent because it is familial 10% of the time. The disease may be hereditary and multifactorial. It may occur by itself in a previously healthy individual. However, many disease states have been reported in association with moyamoya disease, including the following: 1) Immunological - Graves disease/thyrotoxicosis 2) Infections - Leptospirosis and tuberculosis 3) Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus 4) Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease 5) Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, 6)cranial trauma, radiation injury, parasellar tumors, and hypertension etc. These associations may not necessarily be causative but do warrant consideration due to impact on treatment.(Mainly neurosurgical operation.) The incidence of moyamoya disease is highest in Japan. The prevalence of MMD is 1 person per 100,000 population. The prevalence and incidence of moyamoya disease in Japan has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively. With regard to sex, the female-to-male ratio is 1.4:1. A bimodal peak of incidence is noted, with symptoms occurring either in the first decade(5-10yr) or in the third and fourth decades (30-40yr)of life. Mortality rates of moyamoya disease are approximately 10% in adults and 4.3% in children. Death is usually from hemorrhage. In aspect of life insurance, MR is 1700%, EDR is 16 per 1000 persons. Children and adults with moyamoya disease (MMD) may have different clinical presentations. The symptoms and clinical course vary widely from asymptomatic to transient events to severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present. Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. Recently increasing diagnosis of MMD with MRI, followed by surgical operation is noted. MMD needs to be considered as the "CI" state now in life insurance fields.

• Clinical and Experimental Pediatrics
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• v.58 no.11
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• pp.415-420
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• 2015

Purpose: Bronchopulmonary dysplasia (BPD) is characterized by inflammation with proteolytic damage to the lung extracellular matrix. The results from previous studies are inconsistent regarding the role of proteinases and antiproteinases in the development of BPD. The aim of the present study was to investigate whether matrix metalloproteinase (MMP)-8, MMP-9, tissue inhibitor of metalloproteinase (TIMP)-2, and TIMP-1 levels in the serum of preterm infants at birth are related to the development of BPD. Methods: Serum was collected from 62 preterm infants at birth and analyzed for MMP-8, MMP-9, TIMP-2, and TIMP-1 by using enzyme-linked immunosorbent assay. MMPs and TIMPs were compared in BPD (n=24) and no BPD groups (n=38). Clinical predictors of BPD (sex, birth weight, gestational age, etc.) were assessed for both groups. The association between predictors and outcome, BPD, was assessed by using multivariate logistic regression. Results: Sex, birth weight, and mean gestational age were similar between the groups. BPD preterm infants had significantly lower TIMP-2 levels at birth compared with no BPD preterm infants ($138.1{\pm}23.0ng/mL$ vs. $171.8{\pm}44.1ng/mL$, P=0.027). No significant difference was observed in MMP-8, MMP-9, and TIMP-1 levels between the two groups. Multivariate logistic regression analysis indicated that the TIMP-2 levels were predictive of BPD after adjusting for sex, birth weight, gestational age, proteinuric preeclampsia, and intraventricular hemorrhage (${\beta}=-0.063$, P=0.041). Conclusion: Low TIMP-2 serum levels at birth may be associated with the subsequent development of BPD in preterm infants.

• Clinical and Experimental Pediatrics
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• v.54 no.6
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• pp.246-252
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• 2011

Purpose: The outcomes of small for gestational age (SGA) infants especially in extremely low birth weight infants (ELBWIs) are controversial. This study evaluated the mortality and morbidity of ELBWIs, focusing on whether or not they were also SGA. Methods: The medical records of 415 ELBWIs (birth weight<1,000 g), who were inborn and admitted to the Samsung Medical Center neonatal intensive care unit from January 2000 to December 2008, were reviewed retrospectively. Mortality and morbidities were compared by body size groups: very SGA (VSGA), birth weight ${\leq}$3rd percentile; SGA, 3rd to 10th percentile; and appropriate for gestational age (AGA) infants, >10th percentile for gestational age. For gestational subgroup analysis, groups were divided into infants with gestational age ${\leq}24^{+6}$ weeks (subgroup I), $25^{+0}$ to $26^{+6}$ weeks (subgroup II), and ${\geq}27^{+0}$ weeks (subgroup III) Results: Gestational age was $29^{+2}{\pm}2^{+6}$ weeks in the VSGA infants (n=49), $27^{+5}{\pm}2^{+2}$weeks in the SGA infants (n=45), and $25^{+4}{\pm}1^{+4}$ weeks in AGA infants (n=321). Birth weight was $692{\pm}186.6$ g, $768{\pm}132.9$ g, and $780{\pm}142.5$ g in the VSGA, SGA, and AGA groups, respectively. Cesarean section rate and maternal pregnancy-induced hypertension were more common in the VSGA and SGA than in AGA pregnancies. However, chorioamnionitis was more common in the AGA group. The mortalities of the lowest gestational group (subgroup I), and also of the lower gestational group (subgroup I+II) were significantly higher in the VSGA group than the SGA or AGA groups (P=0.020 and P=0.012, respectively). VSGA and SGA infants showed lower incidence in respiratory distress syndrome, ductal ligation, bronchopulmonary dysplasia, intraventricular hemorrhage than AGA group did. However, by multiple logistic regression analysis of each gestational subgroup, the differences were not significant. Conclusion: Of ELBWIs, extremely SGA in the lower gestational subgroups, had an impact on mortality, which may provide information useful for prenatal counseling.

• Journal of Korean Neurosurgical Society
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• v.30 no.2
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• pp.207-210
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• 2001

Objective : The prognosis of spontaneous intracerebral hemorrhage often depends on initial neurologic condition, size and location of hemorrhage and associated intraventricular hemorrhage. However, age of patient, coagulation state and other associated vascular diseases may also play a role when present. In recent years, the geriatric population has been increasing. The age distribution of the patients with intracerebral hemorrhages also has been increased, accordingly. However, such patients, especially when associated with poor initial conditions often tend to be managed rather conservatively. The authors analyzed retrospectively on forty-five patients with spontaneous intracerebral hemorrhage over the seventies with poor initial condition to find out whether there exists a difference of outcome between surgery and non-surgery group. Material and Method : A total of 45 patients over seventies with spontaneous intracerebral hemorrhage with Glasgow Coma Scale(GCS) 4-8 treated over last six years were included. The validity of surgical management for these patients as well as clinical variables which might have been operated on the outcome of these patients were evaluated. The Glasgow Outcome Scale(GOS) after three months was used for comparison of outcome. Results : In surgical group(19 cases), mean age was 74.5 years old, mean hematoma volume 67.2ml and mean GCS score 5.7 points. In nonsurgical group(26 cases), mean age was 79.3 years old, mean hematoma volume 32.1ml, and mean GCS score 6.8 points. Mortality rate in surgical group was 47.4%(9 patients), including 2 cases of post-operative rebleeding, while that in nonsurgical group was 46.2%. However, when patients with initial GCS 4-6 points and over 30ml in hematoma volume were regrouped, mortality rate in surgical group was 46.2%, whereas mortality rate in nonsurgical group was 66.7%. Conclusion : It is concluded that the mortality rate is much low in surgery group with initial GCS less than 6 points and hematoma volume over 30cc. There was no significant difference of outcome in patients with basal ganglia and thalamic hemorrhage. However, surgical treatment lowered the mortality and morbidity rate in patients with subcortical and cerebellar hemorrhage.

• Journal of Korean Neurosurgical Society
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• v.62 no.6
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• pp.643-648
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• 2019

Objective : Shunt-dependent hydrocephalus (SdHCP) is a well-known complication of aneurysmal subarachnoid hemorrhage (SAH). The risk factors for SdHCP have been widely investigated, but few risk scoring systems have been established to predict SdHCP. This study was performed to investigate the risk factors for SdHCP and devise a risk scoring system for use before aneurysm obliteration. Methods : We reviewed the data of 301 consecutive patients who underwent aneurysm obliteration following SAH from September 2007 to December 2016. The exclusion criteria for this study were previous aneurysm obliteration, previous major cerebral infarction, the presence of a cavum septum pellucidum, a midline shift of >10 mm on initial computed tomography (CT), and in-hospital mortality. We finally recruited 254 patients and analyzed the following data according to the presence or absence of SdHCP : age, sex, history of hypertension and diabetes mellitus, Hunt-Hess grade, Fisher grade, aneurysm size and location, type of treatment, bicaudate index on initial CT, intraventricular hemorrhage, cerebrospinal fluid drainage, vasospasm, and modified Rankin scale score at discharge. Results : In the multivariate analysis, acute HCP (bicaudate index of ${\geq}0.2$) (odds ratio [OR], 6.749; 95% confidence interval [CI], 2.843-16.021; p=0.000), Fisher grade of 4 (OR, 4.108; 95% CI, 1.044-16.169; p=0.043), and an age of ${\geq}50years$ (OR, 3.938; 95% CI, 1.375-11.275; p=0.011) were significantly associated with the occurrence of SdHCP. The risk scoring system using above parameters of acute HCP, Fisher grade, and age (AFA score) assigned 1 point to each (total score of 0-3 points). SdHCP occurred in 4.3% of patients with a score of 0, 8.5% with a score of 1, 25.5% with a score of 2, and 61.7% with a score of 3 (p=0.000). In the receiver operating characteristic curve analysis, the area under the curve (AUC) for the risk scoring system was 0.820 (p=0.080; 95% CI, 0.750-0.890). In the internal validation of the risk scoring system, the score reliably predicted SdHCP (AUC, 0.895; p=0.000; 95% CI, 0.847-0.943). Conclusion : Our results suggest that the herein-described AFA score is a useful tool for predicting SdHCP before aneurysm obliteration. Prospective validation is needed.