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OBJECTIVE: to present a new and easy classification of atlanto-axial rotatory fixation (AARF) and to investigate the efficiency of conservative treatment of AARF. BACKGROUND: Although there is a precise definition and diagnostic classification of AARF, there is still significant difficulty in measuring the atlas and axis angles because all of the atlas or axis cannot be seen in a certain 2-dimensional computed tomogram image. In addition, some recent case reports showed that long-term conservative treatment can reduce pediatric AARFs, even that are severe or chronic. METHODS: Fifty-one children with AARF were analyzed retrospectively with new 3-dimensional computed tomogram (3DCT)-based AARF classification; the mean age was 72.7 ± 35.2 months (19-139 months). In the new AARF classification, type 1 was defined as that when the C1C2 angle is not 0° on midline and type 2 as that when the C1C2 angle is 0° on the midline. RESULTS: All 7 children with AARF type 1 were treated successfully only with Halter tractions. Twenty among 44 children with type 2 did not show any difference in improvement compared with not-treated 24 children with type 2. CONCLUSION: The first new AARF classification based on 3DCT appears to be easy to use and even the most severe children with AARF may be managed only with conservative treatment such as long-term Halter traction.
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Articulación Atlantoaxoidea , Luxaciones Articulares , Tortícolis , Articulación Atlantoaxoidea/diagnóstico por imagen , Niño , Preescolar , Tratamiento Conservador , Humanos , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
Pediatric abusive head trauma (AHT) is a serious, repeated child abuse that causes grave brain damage. In Korea, AHT cases have been reported rarely, especially infants. We present 4 cases of AHT in infants diagnosed in our institution during last 2 years. We collected the demographic data, ophthalmologic examination, imaging study, and outcomes. The mean age was 7.2 months, and 2 infants were girls and the others were boys. All four were admitted with no history of head trauma, and among them 2 patients presented with an episode of seizure and respiratory arrest with no history of head trauma. The initial mental status was semi-coma in 3 cases, and stupor in 1 case. There were multiple retinal hemorrhages in both eyes in 2 cases; one had multiple old fractures on the extremities and another child showed multiple skull fractures. All patients underwent emergent surgery for acute bilateral subdural hemorrhages; 3 had craniotomy and another had burr-hole drainage. Two children expired and the other 2 are in vegetative status. The AHT has recently become more frequent in Korea so that neurosurgeons must alert AHT even in infants with head trauma.
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BACKGROUND: The aim of this study was to retrospectively evaluate and analyze the relationships between head circumference percentile (HCP), lumbar puncture pressure (LPP), and cerebrospinal fluid (CSF) space. METHODS: The 88 patients were divided into 3 age groups (group 1, up to 12 months; group 2, 12-36 months; group 3, 36-72 months). RESULTS: In group 1 (n = 40), there was a significant positive correlation of the HCP with the LPP (r =0.414, p =0.008), Evans ratio (r =0.365, p =0.021), and thickness of subdural hygroma (SDHG; r =0.403, p =0.010). Group 2 (n = 29) revealed a significant positive correlation between the LPP and the thickness of SDHG (r =0.459, p =0.012). Group 3 (n = 19) showed no significant correlation among these factors. Overall, age was related with SDHG thickness both in infants and toddlers, while HCP was related with LPP, Evans ratio, and SDHG thickness only in infants, and LPP was related with SDHG thickness only in toddlers. CONCLUSION: We suggest that increased cerebrospinal space and pressure may result in compensatory enlargement of head circumference only in the infant period, and the SDHG thickness decreases with age during the infant and toddler phases.
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Adaptación Fisiológica , Cefalometría , Ventrículos Cerebrales/diagnóstico por imagen , Presión Intracraneal , Punción Espinal , Factores de Edad , Líquido Cefalorraquídeo , Preescolar , Discapacidades del Desarrollo/complicaciones , Femenino , Humanos , Lactante , Recién Nacido , Hipertensión Intracraneal/complicaciones , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Convulsiones/complicaciones , Efusión Subdural/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: The aim of this paper was to investigate the local reactions due to the melting of absorbable plates and screws after trimming through smoothing by multiple sonic activation procedures (MSAP). BACKGROUND: Drilling or smoothing by MSAP is performed for the trimming of the absorbable plates and screws for cranial fixation in children. Compared to drilling, smoothing by MSAP is suspected to more commonly cause local tissue reactions; thus, the issue was examined herein. METHODS: For 90 children with smoothing by MSAP and 83 children with drilling procedures who underwent cranial fixation using absorbable plates and screws, the type, time of onset, and incidence of the local reactions were investigated. RESULTS: Swelling was the most common reaction, followed by inflammatory reactions with itching, heating, skin-penetrating drainage, and pus formation. The mean time of onset of the local reaction was 10.0 ± 2.3 months after surgery. Local reactions occurred in 30% of the children in the group with smoothing by MSAP, and in 7.2% of the children in the group with drilling, showing a statistically significant difference between the two groups (p < 0.001). CONCLUSION: It was concluded that drilling rather than smoothing by MSAP to change the shapes of the absorbable plates and screws in cranial fixation can reduce the local reactions.
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Implantes Absorbibles , Materiales Biocompatibles/efectos adversos , Placas Óseas , Tornillos Óseos , Craneosinostosis , Inflamación/etiología , Preescolar , Anomalías Craneofaciales/cirugía , Craneosinostosis/complicaciones , Craneosinostosis/cirugía , Femenino , Humanos , Masculino , Complicaciones Posoperatorias/etiología , Cráneo/cirugíaRESUMEN
OBJECTIVE: Skull fractures caused by birth injury are mainly compression and linear fractures, but the incidence is known to be rare. It has been recently suggested, however, that greenstick fractures (GSFs) are more common than compression or linear fractures that are detected through 3-dimensional reconstructed computed tomography. Therefore, this study was conducted to determine the correlation of GSF with perinatal factors, the accompanying head injury factors, and the long-term outcomes in neonates. METHODS: A retrospective study was conducted on correlations between GSF and perinatal and head injury factors for 295 neonates. In 160 neonates, the correlation with long-term outcome was investigated. RESULTS: In 295 neonates, 47 cases had 3 or fewer GSFs, 66 cases had 4-6, 82 cases had 7-9, and 100 cases had 10 or more. The number of GSFs was significantly associated with gestational age and mode of delivery among the perinatal factors, with brain swelling and cephalohematoma among the head injury factors. It was also associated with long-term outcomes. The cut-off value of the number of GSFs for a poor outcome by the area under the receiver operating characteristic curve was 6 or greater. CONCLUSIONS: It was concluded that GSF most commonly occurs as a birth-related skull fracture and that birth-related GSF is associated with perinatal factors, birth-related head injuries, and deterioration of long-term development. Therefore, efforts should be made to prevent GSF at birth.
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Traumatismos del Nacimiento/complicaciones , Trastornos del Neurodesarrollo/epidemiología , Trastornos del Neurodesarrollo/etiología , Fracturas Craneales/etiología , Desarrollo Infantil , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Estudios RetrospectivosRESUMEN
OBJECTIVE: The new direct gradual cranial expansion surgical technique has been used to treat children with postshunt microcephaly and slit ventricle syndrome. To evaluate the feasibility of this new surgical treatment, we studied intracranial pressure (ICP) in microcephalic children with developmental delay. METHODS: Mean ICP, age, sex, head size, and developmental assessments were compared in 24 microcephalic children with developmental delay who had had continuous ICP monitoring. RESULTS: Children studied included 9 boys and 15 girls with a mean age of 4.9 ± 2.0 years. Mean ICP was 18.7 ± 8.6 mm Hg. Children with high ICP had significantly lower age and higher B wave ratios than children with low ICP. There were no statistically significant differences in developmental scores and head sizes between children with high ICP and children with low ICP. In multiple linear regression analysis, we observed significantly increased risk of mean ICP elevation by B wave ratio and developmental score and decreased risk of mean ICP elevation by age, but not significantly increased risk of mean ICP elevation by head circumferences (z score). CONCLUSIONS: Our findings suggest that a portion of microcephalic children with developmental delay have high ICP that cannot be expected from head sizes, and high ICP has decreasing tendency with age.
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Discapacidades del Desarrollo/fisiopatología , Hipertensión Intracraneal/fisiopatología , Microcefalia/fisiopatología , Cefalometría , Niño , Preescolar , Discapacidades del Desarrollo/complicaciones , Electrodos Implantados , Estudios de Factibilidad , Femenino , Humanos , Hipertensión Intracraneal/complicaciones , Presión Intracraneal/fisiología , Masculino , Microcefalia/complicaciones , Microcefalia/cirugía , Monitoreo Ambulatorio/instrumentación , Monitoreo Fisiológico/instrumentación , Procedimientos Neuroquirúrgicos/métodos , Factores de RiesgoRESUMEN
OBJECTIVE: The objective of this study was to reinterpret the neurodevelopmental prognostic factors that are associated with birth head injury by performing a long-term follow-up. METHODS: Seventy-three neonates with head injuries were retrospectively analyzed after a duration of 10.0±7.3 years to determine the correlations between perinatal factors, including gender, head circumference, gestational age, body weight, and mode of delivery, and head injury factors from radiologic imaging with social, fine motor, language, and motor developmental quotients. RESULTS: There was a statistically significant difference between perinatal factors and head injury factors with respect to head circumference, body weight, gestational age, mode of delivery, Apgar scores at 1 min, cephalohematoma, subdural hemorrhage, subarachnoid hemorrhage, and hypoxic injury, but no direct correlation by regression analysis was observed between perinatal factors and developmental quotients. Of the head injury factors, falx hemorrhage showed a significant indirect relationship with the language and motor developmental quotients. Mode of delivery, subgaleal hematoma, cephalohematoma, greenstick skull fracture, epidural hemorrhage (EDH), tentorial hemorrhage, brain swelling, and hypoxic injury showed an indirect relationship with social development. CONCLUSION: In terms of perinatal factors and head injury factors, mode of delivery, subgaleal hematoma, cephalohematoma, greenstick skull fracture, EDH, tentorial hemorrhage, falx hemorrhage, brain swelling, and hypoxic injury displayed an indirect relationship with long-term development, and therefore these factors require particular attention for perinatal care.
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OBJECTIVE: Infants are vulnerable to high acoustic noise. Acoustic noise generated by MR scanning can be reduced by a silent sequence. The purpose of this study is to compare the image quality of the conventional and silent T2 PROPELLER sequences for brain imaging in infants. METHODS: A total of 36 scans were acquired from 24 infants using a 3 T MR scanner. Each patient underwent both conventional and silent T2 PROPELLER sequences. Acoustic noise level was measured. Quantitative and qualitative assessments were performed with the images taken with each sequence. RESULTS: The sound pressure level of the conventional T2 PROPELLER imaging sequence was 92.1 dB and that of the silent T2 PROPELLER imaging sequence was 73.3 dB (reduction of 20%). On quantitative assessment, the two sequences (conventional vs silent T2 PROPELLER) did not show significant difference in relative contrast (0.069 vs 0.068, p value = 0.536) and signal-to-noise ratio (75.4 vs 114.8, p value = 0.098). Qualitative assessment of overall image quality (p value = 0.572), grey-white differentiation (p value = 0.986), shunt-related artefact (p value > 0.999), motion artefact (p value = 0.801) and myelination degree in different brain regions (p values ≥ 0.092) did not show significant difference between the two sequences. CONCLUSION: The silent T2 PROPELLER sequence reduces acoustic noise and generated comparable image quality to that of the conventional sequence. Advances in knowledge: This is the first report to compare silent T2 PROPELLER images with that of conventional T2 PROPELLER images in children.
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Aumento de la Imagen/métodos , Imagen por Resonancia Magnética/métodos , Neuroimagen/métodos , Ruido , Seguridad de Equipos , Femenino , Humanos , Lactante , Masculino , Relación Señal-RuidoRESUMEN
OBJECTIVE: The most common birth-associated head injuries during vaginal delivery are cephalhematomas and subgaleal hematomas. Cranial injuries are rarely encountered. The neonate cranium is soft and pliable, and greenstick skull fractures (GSFs) are expected to be more frequent than linear or depressed fractures, but they are extremely difficult to detect with simple skull radiography. As a result, no reports have been issued on this topic to date. Recent reports suggest that technological advances in 3-dimensional (3D) computed tomography (CT) have successfully enhanced the diagnostic accuracy for cranial fractures. The authors researched the types and characteristics of GSFs and the diagnostic accuracy of 3D CT for cranial fractures in neonates. METHODS: The simple skull radiographs and 3D CT images of 101 neonates were retrospectively evaluated and compared with respect to diagnosis of cranial fractures, and skull GSFs were classified on the basis of 3D CT findings into 5 types depending on multiplicity and location. RESULTS: 3D CT detected 88 cases of cranial fractures, that is, 89 GSFs, 4 combined GSFs and linear fractures, and 3 combined GSFs and depressed fractures. The diagnostic rate of 3DCT was 91% and this was significantly higher than the 13% rate of simple skull radiographs (P < 0.001). CONCLUSIONS: GSFs rather than linear fractures were found to account for most cranial injuries among neonates. The diagnostic accuracy of 3D CT was considerably superior than simple skull radiography, but the high radiation exposure levels of 3D CT warrant the need for development of a modality with lower radiation exposure.
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Traumatismos del Nacimiento/diagnóstico por imagen , Procesamiento de Imagen Asistido por Computador , Imagenología Tridimensional , Fractura Craneal Deprimida/diagnóstico por imagen , Fracturas Craneales/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Traumatismos del Nacimiento/clasificación , Traumatismos del Nacimiento/epidemiología , Estudios Transversales , Femenino , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos , Sensibilidad y Especificidad , Fractura Craneal Deprimida/clasificación , Fractura Craneal Deprimida/epidemiología , Fracturas Craneales/clasificación , Fracturas Craneales/epidemiologíaRESUMEN
BACKGROUND: Diastatic skull fractures (DSFs) in children are difficult to detect in skull radiographs before they develop into growing skull fractures; therefore, little information is available on this topic. However, recent advances in 3-dimensional (3D) computed tomography (CT) imaging technology have enabled more accurate diagnoses of almost all forms of skull fracture. The present study was undertaken to document the clinical characteristics of DSFs in children and to determine whether 3D CT enhances diagnostic accuracy. METHODS: Two hundred and ninety-two children younger than 12 years with skull fractures underwent simple skull radiography, 2-dimensional (2D) CT, and 3DCT. Results were compared with respect to fracture type, location, associated lesions, and accuracy of diagnosis. RESULTS: DSFs were diagnosed in 44 (15.7%) of children with skull fractures. Twenty-two patients had DSFs only, and the other 22 had DSFs combined with compound or mixed skull fractures. The most common fracture locations were the occipitomastoid (25%) and lambdoid (15.9%). Accompanying lesions consisted of subgaleal hemorrhages (42/44), epidural hemorrhages (32/44), pneumocephalus (17/44), and subdural hemorrhages (3/44). A total of 17 surgical procedures were performed on 15 of the 44 patients. Fourteen and 19 patients were confirmed to have DSFs by skull radiography and 2D CT, respectively, but 3D CT detected DSFs in 43 of the 44 children (P < 0.001). CONCLUSION: 3D CT was found to be markedly superior to skull radiography or 2D CT for detecting DSFs. This finding indicates that 3D CT should be used routinely rather than 2D CT for the assessment of pediatric head trauma.
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Imagenología Tridimensional , Fracturas Craneales/diagnóstico por imagen , Cráneo/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adolescente , Factores de Edad , Encefalopatías/diagnóstico por imagen , Encefalopatías/epidemiología , Encefalopatías/etiología , Niño , Preescolar , Femenino , Fracturas Abiertas/diagnóstico por imagen , Fracturas Abiertas/epidemiología , Fracturas Abiertas/cirugía , Humanos , Incidencia , Lactante , Masculino , Variaciones Dependientes del Observador , Cráneo/cirugía , Fracturas Craneales/epidemiología , Fracturas Craneales/cirugíaRESUMEN
OBJECTIVE: Among shunt complications, the postshunt slit ventricle (PSSV) and the postshunt craniosynostosis (PSCS) may be managed by shunt valve upgrade and/or cranial expansion surgery. Here, we analyzed 26 children with PSSV, PSCS, or microcephaly who received simple generalized cranial expansion (ie, total calvarial transsutural distraction osteogenesis [TC-TSuDO]). METHODS: Among 254 children with shunt surgery, 26 children received TC-TSuDO. These 26 children included 14 with PSSV, 4 with PSCS, and 8 with both PSSV and PSCS. The mean age of patients who underwent shunt procedures was 8.2 ± 10.6 months, and the mean time interval from shunt surgery to TC-TSuDO was 26.8 ± 29.5 months. The mean age of children at the time of TC-TSuDO was 33.3 ± 30.2 months. We analyzed head circumferences, lumbar puncture pressures, development status, operative factors, and postoperative complications. RESULTS: The mean preoperative head circumference was -2.1 ± 1.9, which increased to -1.4 ± 2.1 (P < 0.001) postsurgically. The mean preoperative lumbar puncture pressure was 26.2 ± 10.7 cm H2O which decreased to 11.9 ± 3.5 cm H2O (P < 0.001) after surgery. The mean operation time was 138 ± 66 minutes. The mean intensive care unit stay was 0.27 ± 0.53 days. There were no mortalities but 2 patients suffered from distractor malfunction and 1 patient showed wound discharge. CONCLUSIONS: We suggest that postshunt complications such as PSSV or PSCS, especially those that are accompanied by increased intracranial pressure or postshunt microcephaly, may be managed for patients with TC-TSuDO, which has been shown to be safe, simple, and effective.
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Craneosinostosis/cirugía , Microcefalia/cirugía , Osteogénesis por Distracción/métodos , Complicaciones Posoperatorias/cirugía , Síndrome del Ventrículo Colapsado/cirugía , Derivación Ventriculoperitoneal , Adolescente , Cefalometría , Niño , Preescolar , Craneosinostosis/diagnóstico por imagen , Femenino , Estudios de Seguimiento , Humanos , Imagenología Tridimensional , Lactante , Hipertensión Intracraneal/diagnóstico por imagen , Hipertensión Intracraneal/cirugía , Masculino , Microcefalia/diagnóstico por imagen , Complicaciones Posoperatorias/diagnóstico por imagen , Síndrome del Ventrículo Colapsado/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: The aim of this study was to analyze age-dependent lumbar puncture pressures (LPPs) measured in 262 children with craniosynostosis to provide information that assists in determining surgery. METHODS: From 1 July 2005 to 30 June 2014, 262 children with craniosynostosis diagnosed at the Department of Neurosurgery, Ajou University Hospital, underwent LPP measurement. These children were compared with respect to age, gender, birth weight, head circumference at the time of birth, gestational age, LPP, and developmental assessments. RESULTS: Changes in LPP in children with craniosynostosis according to age were y = -0.0007x(2) + 0.1327x + 21.678 in all patients overall, y = -0.0003x(2) + 0.1166x + 21.466 in children with single-suture craniosynostosis, and y = -0.002x(2) + 0.248x + 22.55 in patients with multiple and syndromic craniosynostosis, indicating a steady and slow increase from birth until the age of 4 years. Also, 59.9% of the 262 children had LPP greater than 20 cm H2O, but when the LPP cutoff level was adjusted for age, this proportion was 99.2%; the new criterion after the adjusted LPP cutoff level showed that for those with increased LPP, the proportion was 63.4%. CONCLUSIONS: We suggest that LPP increases slowly with age in children with craniosynostosis, increased LPP rates in children with craniosynostosis were higher than previously expected, and the new cutoff level criterion that adjusts for age may be more helpful than a fixed cutoff level for all ages.
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Envejecimiento , Craneosinostosis/fisiopatología , Presión Intracraneal/fisiología , Niño , Preescolar , Craneosinostosis/cirugía , Femenino , Humanos , Lactante , Estudios Longitudinales , Masculino , Estudios Retrospectivos , Punción EspinalRESUMEN
OBJECT Although distraction osteogenesis (DO) requires a secondary procedure in the surgical correction of craniosynostosis, it is relatively simple, requires less transfusion, results in a shorter intensive care unit stay, and is quite safe. Because of these positive factors, various DO techniques have been developed. However, there is disagreement regarding the superiority of DO. The authors reported on a new DO technique, transsutural DO (TSDO), 6 years ago that was performed in 23 patients over a period of 6 months, and it continues to be used at the present time. In this paper the authors report the results of TSDO performed in 285 patients with craniosynostosis over a period of 6 years at a single institution. METHODS TSDO consists of a simple suturectomy of the pathological suture followed by direct distraction of the suturectomy site only. Types of TSDO conducted included sagittal TSDO in 95 patients, bicoronal in 14, unilateral coronal in 57, lambdoid in 26, metopic in 13, multiple in 19, syndromic in 33, and secondary in 28. The mean age (± SD) of the patients was 19.4 ± 23.0 months, and mean follow-up was 39.5 ± 21.0 months. RESULTS The mean operating time was 115 ± 43 minutes, and mean anesthesia time was 218 ± 56 minutes. The mean transfusion volume of red blood cell components was 48 ± 58 ml, and mean transfusion volume of fresh-frozen plasma was 19 ± 35 ml. Total transfusion volume was significantly less in infants younger than 12 months of age and in children with lower lumbar puncture pressures (p < 0.05). Complications included 1 (0.4%) death from postoperative acute pneumonia after a distractor removal operation and 23 (8%) surgical morbidities comprising 10 revisions (3.5%) and 13 early removals of distracters (4.6%). CONCLUSIONS TSDO is a simple, effective, and safe method to use for treating all types of craniosynostosis. Some morbidity was experienced in this study, but it may be attributed to the learning curve of the technique.
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Facial deformity is often seen in infants with deformational plagiocephaly and it usually improves with conservative management. However, we know of few studies of the effect of helmet treatment on the facial skeleton. Our aim therefore was to find out its long-term effects on skull remodelling, and on the shape of the face. Seven beagles wore helmets for seven weeks after birth. Seven study beagles and 3 controls were killed and we measured the length, width, and height of the skulls, maxillas, and mandibles. Statistical analysis showed that the total craniofacial length and skull length did not differ significantly, and skull volumes were similar. Maximal craniofacial, skull, maxillary, and mandibular width were all significantly less in the study group. The maximal craniofacial, maxillary, and mandibular widths were strongly correlated with changes in the skull width, and the width:length ratios of the skulls, maxillas, and mandibles did differ significantly. The skull widths in the study group were significantly smaller, which suggests that a soft moulding helmet may change the growth pattern permanently. The effect of a soft moulding helmet on the lateral aspect of the skull affected the width of the face semipermanently. This modulation in the shape of the skull vault and base may change the shape of the maxilla and mandible, which may serve as a background for the use of helmet treatment to change the facial configuration.
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Huesos Faciales/crecimiento & desarrollo , Dispositivos de Protección de la Cabeza , Animales , Cefalometría , Perros , Cara , Mandíbula , Plagiocefalia no Sinostótica/terapiaRESUMEN
INTRODUCTION: The majority of the present distraction osteogenesis techniques involve local site expansion that only produces localized decompression and affords limited decompression and cosmetic results. We designed a new surgical procedure, total calvarial transsutural distraction osteogenesis (TSuDO). METHODS: We performed total calvarial TSuDO surgical procedures in 21 children. The total calvarial TSuDO method consisted of suturectomy and distraction for the sagittal, bicoronal and bilambdoid sutures. RESULTS: The mean surgery duration was 110 ± 16 min, the mean transfusion volume was 38 ± 45 ml, an average 4.1 ± 0.4 distractors were applied with a mean latency period of 3.3 ± 0.9 days. The mean activation period was 45 ± 18 days with a consolidation period of 54 ± 23 days. There were 3 complication cases of early removal of the distractors: 1 boy with transient 6th cranial nerve palsy accompanied by fever and 2 children with mild pus discharge from the distractor sites. Preoperative lumbar puncture pressures decreased significantly after distractor removal secondary to surgery (p < 0.001). The preoperative cranium size increased significantly (p < 0.001). CONCLUSION: Total calvarial TSuDO is a simple and safe procedure that may produce wide generalized decompression and good cranial configurations that most closely resemble normal skulls.
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Craneosinostosis/cirugía , Osteogénesis por Distracción/métodos , Procedimientos de Cirugía Plástica/métodos , Cráneo/anomalías , Cráneo/cirugía , Preescolar , Craneosinostosis/diagnóstico , Femenino , Humanos , Lactante , Masculino , Resultado del TratamientoRESUMEN
Childhood abducens nerve palsy can occur as a result of trauma, tumour, vasculopathic disease, elevated intracranial pressure, infection, inflammation, and congenital or idiopathic causes. The authors present two cases of unilateral abduction deficit secondary to a recent trans-sutural distraction osteogenesis (TSuDO) operation for craniosynostosis. After distractor removal, the problem resolved spontaneously over 2-4 months in both cases. This is a first reported case of sixth nerve palsy as a complication of TSuDO operation.
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Many theories have been postulated to date regarding mechanisms involved in intracranial hypertension in patients with long-term, shunt-induced slit ventricle syndrome (SVS), but it still seems difficult to define this entity more clearly. Many hypotheses have attempted to explain the causes of SVS as chronic or intermittent catheter obstruction, brain compliance change, and ventricular herniation and distortion, but this theory does not explain clearly the reason why extraventricular pressure (EVP) is increased and intraventricular pressure (IVP) is low or frequently negative. The authors attempt to postulate a hypothesis by addressing new concept of capillary absorption laziness which results in dissociation of EVP with IVP. We, the authors, propose a concept of 'capillary absorption laziness', which is a tendency of the brain parenchymal extracellular fluid (ECF) not to be absorbed through the brain parenchymal capillary absorption system (BPCAS) that results from the bypass of ECF to shunt in the low or even negative ECF pressure and IVP. If this continues for a prolonged period, the tendency not to be absorbed through the BPCAS, even when the IVP and extracellular fluid pressures increases more than the intracranial pressure (ICP), may be established. This leads to situations of the brain such as parenchymal accumulation of the ECF which results in brain edema or swelling, and eventually distortion or herniation which can act as a functional obstruction and consequent dissociation between the IVP and EVP. Hypothesis of capillary absorption laziness may explain several common phenomena of the SVS such as low or even negative IVP in coexistence with high EVP and high ICP, and in these cases, we expect serious complications of SVS such as brain distortion and herniation. From this hypothesis we attempt to find new shunt management protocols to prevent long-term shunt induced complications.
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Derivaciones del Líquido Cefalorraquídeo , Hidrocefalia/complicaciones , Hipertensión Intracraneal/complicaciones , Síndrome del Ventrículo Colapsado/complicaciones , Humanos , Hidrocefalia/cirugíaRESUMEN
BACKGROUND: For the treatment of traumatic brain edema, an efficient modality has not yet emerged. There have been many studies to date which have reported the employment of low-frequency ultrasound for blood-brain barrier disruption (BBBD). However, the authors have observed that low-intensity ultrasound increases water permeability without cellular damage in cartilage cells. We have therefore attempted to observe the effects of applying this low-intensity ultrasound to an experimental animal model. METHODS: A traumatic brain injury rat model was established according to the weight drop method developing the traumatic brain edema. The degree of BBBD was measured by the changes in the water content and spectrophotometric absorbance of Evans blue dye in the cerebrum after low-frequency ultrasound. RESULTS: The cerebral water content levels showed that the BBBD gradually increased after impact and thereafter decreased after 6 h. After low-frequency ultrasound exposure, the values of water content and spectrophotometric absorbance of Evans blue dye were the lowest at 0 h, and were increased at 2 and 5 h of ultrasonic exposure (after impact). CONCLUSION: We suggest that traumatic brain edema in the rat model may be alleviated by low-frequency ultrasound, and low-frequency ultrasound might be proposed as a novel treatment modality for brain edema.
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Edema Encefálico/metabolismo , Edema Encefálico/terapia , Lesiones Encefálicas/metabolismo , Lesiones Encefálicas/terapia , Terapia por Ultrasonido/métodos , Animales , Barrera Hematoencefálica/metabolismo , Encéfalo/irrigación sanguínea , Encéfalo/metabolismo , Edema Encefálico/cirugía , Lesiones Encefálicas/cirugía , Colorantes/farmacocinética , Craniectomía Descompresiva , Modelos Animales de Enfermedad , Diseño de Equipo , Azul de Evans/farmacocinética , Microburbujas , Microcirculación/fisiología , Ratas , Ratas Sprague-Dawley , Agua/metabolismoRESUMEN
BACKGROUND: Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. In particular, coronal synostosis evidences a higher tendency to be genetically caused, and TWIST1 and FGFR3 have been identified as major causative genes. The authors analyzed the clinical and molecular characteristics of Korean patients with coronal synostosis in this study. METHODS: Forty-three Korean patients with unicoronal or bicoronal synostosis were included in this study. All samples were first screened for TWIST1 and FGFR3 mutation hot spots, and the negative samples were subsequently screened for FGFR2. The patients' clinical features were analyzed and compared. RESULTS: Seven sequence alterations (six in TWIST1 and one in FGFR3) were identified in 11 patients (25.6 percent). Three novel TWIST1 mutations were detected, and p.P250R was the only mutation in FGFR3. Bicoronal cases evidenced a much higher mutation detection rate (52.9 percent) than unicoronal cases (7.7 percent). In the TWIST1 group, five patients had Saethre-Chotzen syndrome, and one was nonsyndromic. In the FGFR3 group, four patients had Muenke syndrome, and one was nonsyndromic. The majority of associated anomalies, with the exception of psychomotor retardation and Chiari malformation, were detected more frequently in TWIST1 patients than in FGFR3 p.P250R patients. CONCLUSIONS: This study is, to the best of the authors' knowledge, the first to illustrate the frequency and spectrum of mutations in TWIST1 and FGFR3 in Korea. Considering that molecular diagnosis techniques can prove helpful in providing adequate genetic counseling and guidance, genetic screening for TWIST1 and FGFR3 p.P250R in cases of coronal synostosis is recommended.
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Craneosinostosis/genética , Proteínas Nucleares/genética , Receptor Tipo 3 de Factor de Crecimiento de Fibroblastos/genética , Proteína 1 Relacionada con Twist/genética , Acrocefalosindactilia/genética , Niño , Preescolar , Femenino , Pruebas Genéticas , Humanos , Lactante , Corea (Geográfico) , Masculino , Mutación , Receptor Tipo 2 de Factor de Crecimiento de FibroblastosRESUMEN
OBJECTIVE: The purpose of this study was to elucidate the anatomical development of physiologic suture closure processes in infants using three dimensional reconstructed computed tomography (CT). METHODS: A consecutive series of 243 infants under 12 months of age who underwent three dimensional CT were included in this study. Four major cranial sutures (sagittal, coronal, lambdoidal and metopic suture) were classified into four suture closure grades (grade 0=no closure along the whole length, grade 1=partial or intermittent closure, grade 2=complete closure with visible suture line, grade 3=complete fusion (ossification) without visible suture line), and measured for its closure degree (suture closure rates; defined as percentage of the length of closed suture line divided by the total length of suture line). RESULTS: Suture closure grade under 12 months of age comprised of grade 0 (n=195, 80.2%), grade 1 (n=24, 9.9%) and grade 2 (n=24, 9.9%) in sagittal sutures, whereas in metopic sutures they were grade 0 (n=61, 25.1%), grade 1 (n=167, 68.7%), grade 2 (n=6, 24%) and grade 3 (n=9, 3.7%). Mean suture closure rates under 12 months of age was 58.8% in metopic sutures, followed by coronal (right : 43.8%, left : 41.1%), lambdoidal (right : 27.2%, left : 25.6%) and sagittal sutures (15.6%), respectively. CONCLUSION: These quantitative descriptions of cranial suture closure may help understand the process involved in the cranial development of Korean infants.