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ABSTRACT: 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.
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.
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.
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.
Plastic and reconstructive surgery 05/2012; 129(5):814e-21e. · 2.74 Impact Factor
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ABSTRACT: 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).
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).
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.
These quantitative descriptions of cranial suture closure may help understand the process involved in the cranial development of Korean infants.
Journal of Korean Neurosurgical Society 01/2012; 51(1):31-6. · 0.60 Impact Factor
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ABSTRACT: Distraction osteogenesis for the treatment of craniosynostosis is becoming more widely used as it is simple, there are less transfusions, and a decreased incidence of complications, although a secondary procedure for the removal of the distractors is necessary. However, to date all previous procedures have still been complicated. The authors present a novel trans-sutural distraction osteogenesis method (TSuDO) for the treatment of all types of craniosynostosis.
The TSuDO consisted of simple suturectomy of the pathologic suture followed by direct distraction of the suturectomy site only. Types of TSuDO conducted were sagittal TSuDO in 6 patients, coronal TSuDO in 5 patients, unilateral coronal TSuDO in 8 patients, lambdoid TSuDO in 2 patients, and metopic TSuDO in 1 patient (total = 22). Mean age was 9.3 ± 12.7 months.
The mean operation time was 143.6 ± 50.2 min, and mean total transfusion volume of blood components was 131.1 ± 78.3 ml. Immediate correction of the abnormal head contour after distraction was observed in all patients, and no complications were encountered except for 1 patient whose distractor malfunctioned and 2 who showed prolonged discharges from the pin sites (controlled by antibiotics).
TSuDO is a simple, effective, and safe method for the treatment of all types of craniosynostosis, and is especially effective for the correction of unilateral coronal craniosynostosis.
Pediatric Neurosurgery 11/2011; 47(3):167-75. · 0.70 Impact Factor
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ABSTRACT: Many theories have been postulated to date regarding the mechanisms involved in the absorption of the intracranial arterial blood flow energy in the intracranial space, but it is as yet nor clearly defined. The blood flow energy that is transmitted from the heart formulates the cerebrospinal fluid (CSF) pulsatile flow, and is known to constitute the major energy of the CSF flow, while the bulk flow carries only small energy. The intracranial space that is enclosed in a solid cranium and is an isolate system as in the Monroe-Kellie doctrine, and the authors propose to re-analyze the Monroe-Kellie doctrine concept in terms of energy transfer and dissipation of the Windkessel effect. We propose that the large blood flow energy that initiates in the heart is transferred in order of precedence to the arteries, arterioles, brain parenchyma, and finally to CSF within the cranium, in which the energy is confined and unable to be transferred, so that the final transfer of energy to the CSF pulsatile flow is self-dissipated in terms of direction and chronology in CSF pulsatile flow. In order for the CSF pulsatile flow that is transferred from arterial blood flow energy to be dissipated in the intracranial space, this cannot be explained with bulk flow energy in any perspective, since the pulsatile flow kinetic energy is greater than the bulk flow kinetic energy by at least a 100-fold. The pulsatile flow energy within the closed intracranial space cannot be transferred and is confined, as postulated by the Monroe-Kellie doctrine, and therefore the authors propound that the pulsatile flow dissipates by itself. The dissipation of the CSF pulsatile flow kinetic energy will be in all directions in a diffuse and random manner, and is offset by the CSF flow energy vector due to the CSF mixing process. Such energy dissipation will lead to maintenance of low CSF flow energy, which will result in maintaining low intracranial pressure (ICP), and sufficient brain perfusion. It is our opinion that our hypothesis will be able to explain the decreasing offsetting effect of arterial pulsation in chronic obstructive hydrocephalus, and the mechanisms for the ventricular dilatation in communicating hydrocephalus without changes in the mean ICP, and therefore highly justifying our hypothesis.
Medical Hypotheses 03/2011; 76(6):884-6. · 1.39 Impact Factor
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ABSTRACT: Increased cell mass is one of the characteristics of senescent cells, but this event has not been clearly defined. When subcellular organellar mass was estimated with organelle-specific fluorescence dyes, we observed that most membranous organelles progressively increase in mass during cell senescence. This increase was accompanied by an increase in membrane lipids and augmented expression of lipogenic enzymes, such as fatty acid synthase (FAS), ATP citrate lyase, and acetyl-CoA carboxylase. The mature form of sterol regulatory element-binding protein (SREBP)-1 was also elevated. Increased expression of these lipogenic effectors was further observed in the liver tissues of aging Fischer 344 rats. Ectopic expression of mature form of SREBP-1 in both Chang cells and primary young human diploid fibroblasts was enough to induce senescence. Blocking lipogenesis with FAS inhibitors (cerulenin and C75) and via siRNA-mediated silencing of SREBP-1 and ATP citrate lyase significantly attenuated H(2)O(2)-induced senescence. Finally, old human diploid fibroblasts were effectively reversed to young-like cells by challenging with FAS inhibitors. Our results suggest that enhanced lipogenesis is not only a common event, but also critically involved in senescence via SREBP-1 induction, thereby contributing to the increase in organelle mass (as a part of cell mass), a novel indicator of senescence.
Journal of Biological Chemistry 09/2010; 285(38):29069-77. · 4.77 Impact Factor
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ABSTRACT: The currently employed technique of distraction osteogenesis (DO) for the surgical treatment of craniosynostosis (CS) is commonly performed due to advantages such as shorter operation time, less bleeding and thus less need for transfusions and a lower risk of infections. Several surgical techniques for the various types of CS have been reported, but a uniform surgical method is as yet unavailable.
We compared 23 patients who underwent rotating DO (RDO) with 15 patients who received conventional craniotomy and remodeling (CR). RDO consisted of suturectomy of the pathologic suture and resection of the bone flap to allow wide suture separation for distraction and open-door rotation.
The mean operation duration in the RDO group was 255.9 +/- 97.8 min, which was significantly shorter than the 414.0 +/- 106.9 min for the CR group (p = 0.0001). Perioperative complications in the RDO group consisted of 2 cases of distractor breakage and 2 cases of minimal pus discharge, while in the CR patients there was 1 case of postoperative epidural hematoma and 1 case of spontaneous bone fracture.
We suggest that RDO may be a valid and efficient method for treating children with CS by DO by expanding the intracranial volume and correcting abnormal skull contour shapes.
Pediatric Neurosurgery 08/2010; 46(2):89-100. · 0.70 Impact Factor
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ABSTRACT: Diverse metabolic alterations, including mitochondrial dysfunction, have often been reported as characteristic phenotypes of senescent cells. However, the overall consequence of senescent metabolic features, how they develop, and how they are linked to other senescent phenotypes, such as enlarged cell volume, increased granularity, and oxidative stress, is not clear. We investigated the potential roles of glycogen synthase kinase 3 (GSK3), a multifunctional kinase, in the development of the metabolic phenotypes in cell senescence. The inactivation of GSK3 via phosphorylation is commonly observed in diverse cell senescences. Furthermore, subcytotoxic concentration of GSK3 inhibitor was sufficient to induce cellular senescence, accompanied by augmented anabolism, such as enhanced protein synthesis, and increased glycogenesis and lipogenesis, in addition to mitochondrial dysfunction. Anabolism was accomplished through glycogen synthase, eIF2B, and SREBP1. These metabolic features seem to contribute to an increase in cellular mass by increasing glycogen granules, protein mass, and organelles. Taken together, our results suggest that GSK3 is one of the key modulators of metabolic alteration, leading the cells to senescence.
Annals of the New York Academy of Sciences 07/2010; 1201:65-71. · 3.15 Impact Factor
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ABSTRACT: The Muenke syndrome (MS) is characterized by unicoronal or bicoronal craniosynostosis, midfacial hypoplasia, ocular hypertelorism, and a variety of minor abnormalities associated with a mutation in the fibroblast growth factor receptor 3 (FGFR3) gene. The birth prevalence is approximately one in 10,000 live births, accounting for 8-10% of patients with coronal synostosis. Although MS is a relatively common diagnosis in patients with craniosynostosis syndromes, with autosomal dominant inheritance, there has been no report of MS, in an affected Korean family with typical cephalo-facial morphology that has been confirmed by molecular studies. Here, we report a familial case of MS in a female patient with a Pro250Arg mutation in exon 7 (IgII-IGIII linker domain) of the FGFR3 gene. This patient had mild midfacial hypoplasia, hypertelorism, downslanting palpebral fissures, a beak shaped nose, plagio-brachycephaly, and mild neurodevelopmental delay. The same mutation was confirmed in the patient's mother, two of the mother's sisters and the maternal grandfather. The severity of the cephalo-facial anomalies was variable among these family members.
Journal of Korean medical science 07/2010; 25(7):1086-9. · 0.84 Impact Factor
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ABSTRACT: Vaginal delivery is accomplished by the force of the labor overcoming the resistance forces of birth canal. During this process, the fetal head passes through the birth canal and the skull receives pressure on the lateral aspect, resulting in molding, the convex shaping of the cranium. Also, the infant's skull is compressed by the mother's pelvic bony structures. These forces may lead to skull fractures and brain injuries. The hypothesis by the authors is that many skull fractures of the newborn present as incomplete fractures. The bony skull of the newborn is histologically primary bone tissue and which is incomplete in its ossification process. During birth the pressure forces upon the newborn's skull is gradual in one direction, rather than a sudden impact, and therefore it is thought that the skull fracture would be an incomplete fracture. However, it is very hard to ascertain the presence of incomplete fractures especially in incompletely ossified skulls with plain X-ray studies, and therefore it is possible that the real incidence of skull fractures in the newborn are higher than reported in the current and past literature. It is also probable that the external forces upon the skull that are sufficient to cause skull fractures, would also lead to significant brain injury more frequently than actually observed, and subsequently contribute to development of many brain disease later in children. The authors of this study propose that very close examination should be conducted to find incomplete fracture, and increased efforts should be made to establish the presence of possible accompanied brain injuries in babies with incomplete skull fracture. The definitive diagnosis and treatment, as well as close follow up of patients with brain injury will assist the clinician in determining the causes of neurological diseases especially in those with previously unknown etiologies, which may be due to birth injuries. Assistance may be also afforded in the early treatment and prevention of such conditions.
Medical Hypotheses 12/2009; 74(5):898-900. · 1.39 Impact Factor
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ABSTRACT: Meningioangiomatosis (MA) is a rare congenital tumor that occurs mostly in 5-15 year old children. There have been only 5 cases previously reported that described the cystic nature within these tumors. We present a case of a MA accompanied by a separate macrocyst. A normally developed 2 year-old female patient presented with partial and generalized seizures. The brain computerized tomogram and magnetic resonance imaging revealed the presence of a calcified mass accompanied by a cyst in the right parietal area, surrounded by low density and high attenuation edema and hemorrhage. Upon right parietal craniotomy, a 1.6 cm x 1.2 cm x 0.5 cm sized plate-like, gray-white, slightly hard mass was seen and it was completely excised. Approximately 1 cm from the mass in the anterior lateral direction, a cyst was found and subsequent biopsy of the cyst wall revealed no tumor tissue, and therefore the cyst was not removed. Pathologic report demonstrated the meningioangiomatosis. Follow up examination 2 years later showed no recurrence of the tumor, and there was no evidence of neurological deficits. Authors suggest that cysts that arise in the surrounding tissues of tumors may not be tumor cysts, and do not require surgical removal.
Journal of Korean Neurosurgical Society 09/2009; 46(3):252-6. · 0.60 Impact Factor
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ABSTRACT: We aimed to identify clinico-radiological risk factors that may predict unfavorable neurological outcomes in traumatic brain injury (TBI), and to establish a guideline for patient selection in clinical trials that would improve neurological outcome during the early post TBI period.
Initial clinico-radiological data of 115 TBI patients were collected prospectively. Regular neurological assessment after standard treatment divided the above patients into 2 groups after 6 months : the Favorable neurological outcome group (GOS : good & moderate disability, DRS : 0-6, LCFS : 8-10) and the Unfavorable group (GOS : severe disability-death, DRS : 7-29 and death, LCFS : 1-7 and death).
There was a higher incidence of age >/=35 years, low initial GCS score, at least unilateral pupil dilatation, and neurological deficit in the Unfavorable group. The presence of bilateral parenchymal lesions or lesions involving the midline structures in the initial brain CT was observed to be a radiological risk factor for unfavorable outcome. Multivariate analysis demonstrated that age and initial GCS score were independent risk factors. The majority of the Favorable group patients with at least one or more risk factors showed improvement of GCS scores within 2 months after TBI.
Patients with the above mentioned clinico-radiological risk factors who received standard treatment, but did not demonstrate neurological improvement within 2 months after TBI were deemed at risk for unfavorable outcome. These patients may be eligible candidates for clinical trials that would improve functional outcome after TBI.
Journal of Korean Neurosurgical Society 02/2009; 45(2):90-5. · 0.60 Impact Factor
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ABSTRACT: Secondary craniosynostosis rarely develops within several months in infants or children after shunt operations in early infancy. However, conventional operations (CO) such as fronto-orbital advancement and total skull reshaping have not been efficient enough to expand the intracranial volume in children with secondary craniosynostosis. Recently, distraction osteogenesis (DO) was reported to be effective in treating most craniosynostosis cases.
We compared 9 children treated with DO and 3 children treated with CO who developed secondary craniosynostosis after shunt operation in early infancy. We measured the preoperative and follow-up head sizes with regard to head circumference, cephalometric intracranial volume, and intracranial volume estimated from the 3-dimensional computed tomogram.
The mean intracranial volumes expanded by 10.5% in the DO group 1 month after surgery and by 13.1% in the CO group on the immediate postoperative day. Further expansion was observed 6 months postoperatively, i.e. 10.3 and 4.7% in the DO and CO groups, respectively. Operation time and anesthesia time were significantly shorter in DO compared to CO patients, and the volumes of the blood transfusions were also less for DO patients.
We suggest that DO is probably more efficient and safer than CO in expanding the intracranial volume in children with secondary craniosynostosis.
Pediatric Neurosurgery 01/2009; 45(6):437-45. · 0.70 Impact Factor
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ABSTRACT: Many theories have been postulated to date regarding mechanisms involved in non-enlargement of the subarachnoid space and enlargement of the ventricles in patients with communicating hydrocephalus, but none have been prove to be definite. Cerebrospinal fluid (CSF) movement is known not to bulk flow but rather pulsatile flow that develops from the energy of the blood flow ejected from the heart, in an isolated system of the intracranial cavity surrounded by a solid skull, as in the Monro-Kellie hypothesis. The authors attempt to explain the mechanisms involved in selective enlargement of the lateral ventricle in patients with communicating hydrocephalus by re-addressing the Monro-Kellie hypothesis with respect to cardiac energy transfer and dissipation by the Windkessel effect. The authors present a concept whereby the large energy of blood flow from the heart that is conveyed to the intracranial artery, arteriole, brain parenchyme, ventricle, and CSF within the confined cranial space as in the Monro-Kellie hypothesis, and which ultimately dissipates to maintain an intracranial energy equilibrium. In the same context, if, for some reason the intracranial equilibrium in the energy transfer and dissipation is changed or disrupted, then structural changes would have to occur to achieve and maintain a new intracranial equilibrium. We postulate that the above described mechanisms are those responsible for the development enlarged of lateral ventricles in patients with communicating hydrocephalus. Structural enlargement of the lateral ventricles in communicating hydrocephalus is a consequence of CSF pathway obstruction and resultantly increased CSF absorption function in the lateral ventricle which markedly increases the pulsatile CSF energy flow returning to the lateral ventricles, thus causing collision of pulsatile CSF flow with the brain parenchyme at the ventricular wall, which subsequently leads to structural enlargement of the lateral ventricles. Also, the collision between the CSF pulsation and brain parenchyme pulsation reduces the Windkessel effect of the brain parenchyme which increases the intracranial artery pulse pressure, which in turn is transmitted to the CSF and increases CSF pulse pressure. This vicious circle results in the high pulse pressure within the lateral ventricle structurally dilating the lateral ventricle. Our theory also explains the relationship between ventricle dilatation and idiopathic intracranial hypertension, venous sinus thrombosis, achondroplasia.
Medical Hypotheses 11/2008; 72(2):174-7. · 1.39 Impact Factor
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ABSTRACT: We present a very rare case of an infant with delayed presentation of spinal cord injury without radiologic abnormality (SCIWORA) after a minor injury.
To emphasize the importance of spinal evaluation with MRI in selected cases, even after minor injuries, especially in infants.
SCIWORA arises mainly in infants and children during accidental trauma or after sport injury. However, it has been very rare for a 6-day-delayed infant SCIWORA after a minor injury.
An infant presented with transient nausea and vomiting after falling from a baby-rocking horse of less than 30-cm height. The patient demonstrated right hemiparesis 6 days later. Plain cervical radiographs and brain and cervical spine computed tomograms (CT) were normal, but the cervical magnetic resonance imaging (MRI) demonstrated a high signal in the T2-weighted image of the lower cervical cord, and a neck collar was applied.
A follow-up cervical MRI 1 month later showed that the high signal of the lower cervical cord had disappeared. Another follow-up cervical MRI 12 months later also showed normal radiographic findings but there still remained mild weakness of the right lower extremities.
The authors present a rare case of infant SCIWORA who developed delayed neurologic symptoms 6 days after a minor injury and suggest that spinal evaluation with MRI could be warranted in the selected case even after minor injuries, especially in infants.
Spine 11/2008; 33(21):E792-4. · 2.08 Impact Factor
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ABSTRACT: Sotos Syndrome is characterized by macrocephaly, overgrowth, and developmental delay, and more than 300 patients have been reported worldwide to date. The authors reviewed the clinical characteristics of 8 patients with Sotos Syndrome in Korea for a new understanding and treatment strategies.
The medical records of a total of eight Korean children with Sotos Syndrome were reviewed. All patients underwent developmental checkup, lumbar punctures for measurement of intracranial pressure (ICP), brain and spine magnetic resonance imaging and computerized tomography.
All 8 patients showed macrocephaly and the characteristic craniofacial features of Sotos Syndrome. Other clinical characteristics shown were overgrowth (7/8), developmental delay (7/8), congenital heart defect (3/8), flat foot (8/8), scoliosis (4/8), spina bifida (8/8), hydrocephalus (4/8), cavum vergae (3/8), and increased subdural fluid collection (5/8). Mean ICP measured via lumbar puncture was 27.35+/-6.25 cm H(2)O (range 20 to 36 cm H(2)O). Two patients received ventriculo-peritoneal shunt, and 1 patient underwent subduro-peritoneal shunt with improvement. Spinal orthosis was applied to 4/5 patients with scoliosis and 4/8 children with flat foot were provided with foot orthosis.
In this first Korean study of 8 Sotos Syndrome patients we demonstrated the presence of spina bifida and increased ICP, which had not been previously described. The authors therefore suggest that all patients with Sotos Syndrome should undergo examination for the presence of spina bifida, and that shunt procedures would improve development and alleviate clinical symptoms.
Journal of Korean Neurosurgical Society 11/2008; 44(4):240-4. · 0.60 Impact Factor
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ABSTRACT: Spontaneous spinal epidural hematoma (SSEH) is rare in children, especially in infants, in whom only 12 cases have been reported. Because of the nonspecificity of presenting symptoms in children, the diagnosis may be delayed. We report herein a case of SSEH in a 20-month-old girl who initially presented with neck pain, and developed lower extremity motor weakness and symptoms of neurogenic bladder 2 weeks prior to admission. The magnetic resonance imaging showed an epidural mass lesion extending from C7 to T4, and the spinal cord was severely compressed by the mass. After emergency decompressive surgery the neurologic function was improved immediately. Two months after surgery, the neurological status was normal with achievement of spontaneous voiding. We suggest that surgical intervention can provide excellent prognosis in case of SSEH in infants, even if surgery delayed.
Journal of Korean Neurosurgical Society 09/2008; 44(2):84-7. · 0.60 Impact Factor
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ABSTRACT: A 7-yr-old boy visited our surgical center with Pfeiffer syndrome type 1, presenting with macrocrania, broad big toe and thumb, exophthalmos, tongue protrusion, malocclusion with midfacial retrusion, mild respiratory difficulty due to minor upper airway obstruction, and developmental delay. He also exhibited anthrophobia with a passive character. The patient was treated with internal monoblock distraction osteogenesis to increase the intracranial and intraorbital volumes, and the nasal and pharyngeal airway spaces using two modular mid-facial internal distractors. For distraction, the latency period was 1 week, the daily activation of 1.0 mm was 20 days (total advancement 20 mm at the midline), and the consolidation period was 3 months. The follow-up computed tomography 12 months after surgery showed expansion of the brain and proper ossification in the distracted area. The patient also showed aesthetically good cranial contours, improved tongue and eyeball protrusion, no respiratory difficulty, and improved learning. We suggest that the internal distraction may last longer than an external type, resulting in a better bone fusion rate and successful expansion of craniofacial bones.
Journal of Korean Medical Science 05/2008; 23(2):342-6. · 0.99 Impact Factor
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ABSTRACT: Most cases of pediatric scalp and skull masses are either congenital or developmental tumors or diseases, and in many patients, these lesions are accompanied by intracranial extension. Past studies pertaining to these conditions are few, and thus clinical information is as yet inadequate.
The authors present 75 pediatric patients with 77 cases of surgically treated scalp and skull masses. The most common pathologic condition was Langerhans cell histiocytosis, followed by epidermal cysts, epidermoid and dermoids, scalp hemangiomas, and neurofibromas, in descending order. Intracranial extension was observed in three cases (4%); two cases were malignant tumors that were skull metastasis of a neuroblastoma and a lymphoma, and one case was encephalocele. Lesions followed up because of possible recurrence were 15 cases of Langerhans cell histiocytosis, eight cases of epidermoid and dermoids, two cases of malignant tumors, and one case each of desmoplastic fibroma, myxoma, fibroid dysplasia, and neurofibroma, totaling 29 cases (39%).
Although the overall incidence of intracranial extension of pediatric scalp and skull masses is very low and the presence of a malignant tumor or metastatic tumor presenting as a scalp and skull mass is rare, such masses enlarge as time progresses, and there exists a possibility of recurrent disease. Therefore, it is suggested that early surgical resection will afford favorable prognosis for the patients, and meticulous follow-up is necessary in some conditions.
Child s Nervous System 05/2008; 24(4):459-65. · 1.54 Impact Factor
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ABSTRACT: Traumatic intracranial pseudoaneurysms in children must be completely secured from the parent artery because of significant morbidity and mortality from the high risk of rebleeding. However, the trapping of the parent artery involves the risk of ischemia changes distal to the trapped artery and the perforator injury. We describe a first case in the literature of successful trapping in the short segment with hydrogel-coated self-expandable coil in a child with a traumatic pericallosal pseudoaneurysm.
A 5-year-old boy was admitted to our institution after enduring a blunt trauma with a stuporous mentality. Initial neuroimaging revealed a small hemorrhage in the corpus callosum with subarachnoid hemorrhage. Two weeks later, computed tomogram showed new callosal hemorrhage and a 4.7 x 5-mm pseudoaneurysm of the right pericallosal artery with mental deterioration and weakness of lower extremity. An endovascular short segmental internal trapping (5 mm) of the right pericallosal artery was conducted to save collateral blood flow, using 2 hydrogel-coated self-expandable coils. The cerebral angiogram of immediately after the trapping and at 3 months' follow-up revealed that the aneurysm had been completely obliterated, with successful maintenance of the distal collateral flow from the contralateral pericallosal artery. The patient recovered with good clinical outcome.
We suggest that short-segment occlusion by a hydrogel-coated self-expandable coil has become a good alternative for the treatment of traumatic pseudoaneurysms in the distal anterior cerebral artery, although the safety and reliability of this procedure is as yet not definitely proven.
Surgical Neurology 05/2008; 69(4):418-22; discussion 422. · 1.67 Impact Factor
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ABSTRACT: Cranial distraction osteogenesis has been applied as a mode of therapy to patients with various types of craniosynostosis. Several minor complications during distraction have been reported in the previous literature, including infection, device exposure, and dislocation and distortion of the device. To our knowledge, this is the first report of 2 cases of spontaneous device breakage during cranial distraction osteogenesis. Two infant patients were diagnosed with shunt-induced microcephalies. The ages of the 2 patients were 8 and 12 months. Their head circumferences were 3 percentiles below that of normal children of the same age. Distraction osteogenesis advancement to the frontal skull and the orbital bar was performed with two distractors. We distracted a total of 7.0 and 14.4 mm at a rate of 0.6-1.5 mm per day before device breakage occurred 7 and 25 days after the distraction activation, respectively. Both patients underwent reoperation to exchange the broken device for further distraction. To prevent further device breakage, we suggest increasing the rate of distraction, placing stronger or more devices, or relieving the scalp retraction pressure probably by extensive dissection of the subcutaneous layers or multiple incisions into the galeal layers.
Pediatric Neurosurgery 02/2008; 44(3):216-20. · 0.70 Impact Factor
