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The epidemiology of patients with traumatic brain injury (TBI) has changed dramatically over recent decades as a result of rapid advances in aging societies. We assessed the influence of age on outcomes of patients with TBI and sought to identify prognostic factors for in-hospital mortality of TBI among elderly patients.Using a nationwide database, we analyzed data from 5651 patients with TBI. Univariate analysis was conducted to compare patient demographics, neurologic status on admission, radiologic findings, systemic complication rates, length of hospital stay, in-hospital mortality, and home discharge rates between elderly and nonelderly groups. Multivariable analysis was conducted to determine prognostic factors for in-hospital mortality among elderly patients.Overall in-hospital mortality was significantly higher in elderly patients (12.8% vs. 19.3%; P0.001). In-hospital mortality of elderly patients with mild TBI increased significantly at7 days after admission, whereas that of elderly patients with moderate or severe TBI was significantly higher immediately after admission. Age (odds ratio [OR], 1.62; P = 0.024), male sex (OR, 1.30; P = 0.004), Japan Coma Scale score on admission (OR, 5.95, P0.001), and incidence of acute subdural hematoma (OR, 1.89; P0.001) were associated with in-hospital mortality in elderly patients with TBI.Elderly patients with TBI showed significantly higher in-hospital mortality. Delayed increases in in-hospital mortality were observed among elderly patients with mild TBI. Level of consciousness on admission was the strongest predictor of in-hospital mortality among elderly patients.

Laparoscopic hepatojejunostomy (HJ) with continuous sutures is commonly performed in laparoscopic pancreaticoduodenectomy (LPD). This study aimed to investigate the long-term surgical outcomes of HJ in LPD. We retrospectively evaluated 103 consecutive patients who underwent pancreaticoduodenectomy via laparoscopic HJ with continuous suturing using multifilament (n = 48) or monofilament-absorbable sutures (n = 47). During follow-up, anastomotic stricture of HJ was identified in 8 (7.8%) patients via balloon enteroscopy-assisted cholangiography. The median time from surgery to confirmation of stricture formation was 7.6 months (range 3.6–19.4). The incidence of HJ stricture was significantly higher in patients with a thin bile duct (diameter

Objectives Infected subdural hematoma (ISH) is a rare type of subdural empyema, with fewer than 50 cases reported to date. Its radiological features have not been adequately described, making diagnosis challenging. At our institution, two adults presented with ISH, which exhibited a characteristic shape on preoperative imaging. Patients and methods This study examined ISH cases and chronic subdural hematoma (CSH) cases that underwent surgery at the Ishikawa Prefectural Central Hospital between January 2016 and March 2018. To distinguish ISH from CSH, we focused on three specific radiological features: the biconvex shape of the hematoma, presence of a high-density region at the lower end of the hematoma on plain computed tomography (CT), and presence of a hyper-intense signal within the hematoma on diffusion weighted imaging (DWI). Results We analyzed 30 ISH (current and previously reported) and 102 CSH cases in our study. We found no statistically significant associations between the hematoma type (ISH or CSH) and the presence of a high-density region at the lower end of the hematoma on plain CT (p = 0.13) or the presence of hyperintensity in the hematoma on DWI (p = 1.00). Conversely, a statistically significant association was found between the hematoma type and the biconvex shape of the hematoma (p Conclusion These results suggest that the shape of the hematoma on imaging provides valuable information that can be used to differentiate ISH from CSH and optimize therapeutic approaches.

Rhabdomyosarcoma is the most common soft-tissue sarcoma affecting children and adolescents. It is defined as a malignant neoplasm characterized by morphologic, immunohistochemical, ultrastructural, or molecular genetic evidence of primary skeletal muscle differentiation, usually in the absence of any other pattern of differentiation. Primary intracranial rhabdomyosarcoma (PIRMS) is an extremely rare neoplasm, with only 60 cases reported in the literature, and generally has poor prognosis with an overall survival of only 9.1 months. The DICER1 gene encodes an RNA endoribonuclease that plays a key role in gene expression regulation through the production of small RNAs. Herein, we report two cases of PIRMS with somatic DICER1 mutation showing morphological and immunohistochemical evidence of primary skeletal muscle differentiation; the two cases share common clinical features, including young age, supratentorial tumor, and onset of intratumoral bleeding. Although methylation profiling was not performed, both cases shared clinical and pathological characteristics in common with recently proposed methylation entity “spindle cell sarcoma with rhabdomyosarcoma-like features, DICER1 mutant (SCS-RMSlike-DICER1)’’. Our cases provide further evidence of the link between primary intracranial sarcoma and DICER1 mutation which may form a distinct entity.

Background and purpose To assess the relationship between plaque volume evaluated by multidetector computed tomographic angiography (MDCT) and in-stent restenosis (ISR) after carotid artery stenting (CAS). Materials and methods From a retrospectively maintained database, data were collected for 52 patients with carotid artery stenosis treated with CAS between 2007 and 2012. We defined ISR of ≥ 50% as a peak systolic velocity ≥ 200 cm/s on echo-duplex scan. Carotid plaques were subdivided into four components according to radiodensity in Hounsfield units (HU) as follows: 600 HU. Risk factors that influenced ISR were compared using univariate and multivariate Cox regression analyses. Results During a median follow-up period of 36 months, ISR of ≥ 50% was detected in five patients (9.6%). In the univariate Cox proportional hazard regression analysis, renal insufficiency, coronary artery disease, total plaque volume, and plaque volumes with radiodensities

Highlights • We report a rare case of adenoma that developed after sigmoidocolocystoplasty. • There were 11 cases of oncogenesis after bladder augmentation in the literature, including our case. • We recommend routine surveillance cytoscopy and cytology for patients after bladder augmentation, although this remains controversial., Introduction We encountered a rare case of tubular adenoma developing after bladder augmentation. We here report our case as well as summarize reports in the literature on adenomas developing after bladder augmentation. Presentation of case A 23-year-old man came to our hospital for routine surveillance cystoscopy. He was born with a lipomyelomeningocele and neurogenic bladder with low bladder compliance, and hence his bladder was routinely emptied by clean intermittent catheterization. He was also treated with anticholinergic agents. However, because the patient’s neurogenic bladder was unstable, he underwent sigmoidocolocystoplasty when he was 8-years old. After the bladder augmentation, he was examined annually by surveillance cystoscopy. On cystoscopy, a 5-mm pedunculated polyp was found on the front side of the sigmoid colon cap. Therefore, we performed snare polypectomy together with electrocoagulation under cystoscopy. The patient’s final diagnosis was tubular adenoma (mild atypia) with no malignancy, as assessed by histopathology. There has been no evidence of recurrence after the polypectomy on routine surveillance cystoscopy. Discussion To the best of our knowledge, there have been 11 cases of adenoma occurring after bladder augmentation reported in the literature, including our present case. There are several carcinogenic pathways associated with colorectal oncogenesis. Adenomas that are larger than 1.0 cm in diameter with a marked villous component have a high risk of oncogenesis. Conclusion We believe that the early detection of carcinoma or adenoma and their treatment at an early stage is crucial. Therefore, we recommend routine surveillance cystoscopy for patients after bladder augmentation.

A boy with myelomeningocele who had sigmoidocolocystoplasty and ureteric reimplantation when 2-years old and normal annual cystoscopies, developed hematuria and abdominal pain with liver dysfunction 14 years postoperatively. Computed tomography showed a tumor on the left side of the augmented bladder, a large lymph node, and large multiple probable metastases in the liver. Cystoscopy 3 months earlier had been normal, but when repeated showed a tumor originating from the augmented sigmoid colon. Biopsy showed undifferentiated sarcoma. Despite chemotherapy, he died 3 months later. The diagnosis at autopsy was undifferentiated sarcoma originating from the sigmoid colon. We report the first case of undifferentiated sarcoma developing 14 years after sigmoidocolocystoplasty for meningomyelocele, and also review the 55 cases of post-bladder augmentation malignancy in the literature.

There are many reports on position-related complications in neurosurgical literature but so far, continuous quantification of the patient's position during the surgery has not been reported. This study aims to explore the utility of a new surgical table system and its software in displaying the patient's body positions during surgery on real-time basis. More than 200 neurosurgical cases were monitored for their positions intra-operatively. The position was digitally recorded and could be seen by all the members in the operating team. It also displayed the three-dimensional relationship between the head and the heart positions. No position-related complications were observed during the study. The system was able to serve as an excellent indicator for monitoring the patient's position. The recordings were analyzed and even used to reproduce or improve the position in the subsequent operations. The novel technique of monitoring the position of the head and the heart of the patients and the operating table planes are considered to be useful during delicate neurosurgical procedures thereby, preventing inadvertent procedural errors. This can be used to quantify various surgical positions in the future and define safety measures accordingly.

Implantation of carmustine-impregnated wafers (Gliadel®) into the tumor resection cavity has demonstrated a survival benefit for patients with malignant glioma. However, some precautions should be taken regarding Gliadel implantation. We report a case of a 63-year-old man with glioblastoma who was implanted with Gliadel after a left temporal lobe tumor had been removed, and who later developed vasospasm of the lenticulostriate artery close to the implanted Gliadel, leading to serious cerebral infarction. Therefore, the implantation of Gliadel in cases where important vessels run close to the resection cavity should be considered with great caution.

Background/purpose We report the current status of patients who underwent augmentation cystoplasty (AC) at least 20 years previously. Methods Surgical history, incidence of urinary tract infection (UTI) and bladder stones, vesicoureteral reflux (VUR), urine cytology, renal function, a colon cancer tumor marker (carcinoembryonic antigen: CEA), and patient outcomes were assessed. Results Forty patients who underwent AC (mean age: 34.4 years; mean follow-up time: 24.3 years) were analyzed. Mean age at AC was 11 years. Incidence of bladder stones was 30%. There were no incidences of carcinoma after AC, and CEA levels were not increased. Ureteral reimplantation (URI) was performed in 21 patients. URI performed at the same time as AC was successful in 14 cases (93%) and unsuccessful in 1 (7%) because of persistent VUR. UTI developed after AC in only 1 patient (2.5%) with persistent VUR. This patient required unilateral nephrectomy 18 years after the AC because of repeated UTIs. Thirty-four patients (85%) were employed, and 4 (10%) were married. Two of the 19 female patients (11%) had experienced pregnancy and delivery. Five patients (13%) had mental disorders. Conclusion Ultra long-term follow-up suggests that AC is a safe procedure with manageable sequelae, although some mental health issues remain. Type of study Case series with no comparison group. Level of evidence Level IV.

Objective: To study the coping pattern of a mother of a child with multiple congenital anomalies (MCAs) based on interview data. Methods: The mother of a child born at Tohoku University Hospital, Japan, who was diagnosed with MCAs at 3 days of age and subsequently underwent surgery at 7 months, was asked to be interviewed. She answered semi structured interviews at the hospital when the child was two years old and again when the child was five years old. Psychological assessment with the State-Trait Anxiety Inventory (STAI) and the Self-rating Depression Scale (SDS) was conducted before each interview. Results: Psychological assessment showed that the mother had high levels of anxiety and depression when the child was two and when the child was five years old. The interview data showed that when the mother first learned that her baby had MCAs, her initial reaction was (1) shock. She then felt (2) Absent-minded. (3) Responsibility to take care of her child. (4) Depression, (5) Defense against acceptance of child’s disease as it is. The mother had this sequence of thoughts and emotions between the times of diagnosis of MCAs up through the time of surgery. In the first stage of coping, the mother showed defense against learning as much as possible about her child’s disease and MCAs. In the second stage of coping, the mother reported (when the child was five years old) that she felt that she had some control over her child’s situation. The mother started to accept her child’s disease as she as possible and learned what would be happened to her child. Conclusion: The mother of the child with MCAs was in a severe psychological condition over at least five years since diagnosis. In order to alleviate the psychological stress on mothers of a child with MCAs or other congenital disease, it is useful to educate the mother so that she can understand her child’s disease. However, it is difficult for parents without medical knowledge to understand a complicated disease such as MCAs. Providers need more training to educate parents with MCAs.

Hyperintense lesions around the resection cavity on magnetic resonance diffusion-weighted imaging (MR-DWI) frequently appear after brain tumor surgery due to the damage of surrounding brain. The putative connection between the lesion and the prognosis for patients with glioblastoma (GBM) was explored. This retrospective study reviewed consecutive sixty-one patients with newly diagnosed GBM. Postoperative MRI was performed within 2 weeks after the initial surgery. We classified the cases into two groups depending on whether DWI hyperintense lesions were observed or not [DWI(+) group and DWI(−) group]. Progression-free survival (PFS) and overall survival (OS) were compared between the two groups. Forty-two patients were identified. The various extents of hyperintense lesions around the resection cavity were observed in 28/42 (66.7 %) cases. In the DWI(+) and DWI(−) groups, median PFS was 10.0 [95 % confidence interval (CI) 8.4–11.5] and 6.7 (95 % CI 4.9–8.5) months, respectively (p = 0.042), and median OS was 18.0 (95 % CI 12.2–23.8) and 17.0 (95 % CI 15.7–18.3) months, respectively (p = 0.254). On multivariate analysis, the presence of DWI hyperintense lesion was more likely to be an independent predictor for 6-month PFS (p = 0.019; HR, 0.038; 95 % CI 0.002–0.582). Tumor recurrence appeared outside the former DWI hyperintense lesion. Hyperintense lesions surrounding the resected GBM on MR-DWI might be a favorable prognostic factor in patients with GBM.

Glioma, the most common primary brain tumor, is characterized by proliferative-invasive growth. However, the detailed biological characteristics of invading glioma cells remain to be elucidated. A monoclonal antibody (clone HMab-1) that specifically and sensitively recognizes the isocitrate dehydrogenase-1 (IDH1) protein carrying the R132H mutation can identify invading glioma cells by immunostaining. To investigate the degree of invasion in gliomas of distinct grades and the proliferative capacity of the invading cells, immunofluorescent staining was conducted using antibodies against IDH1-R132H and Ki-67 on 11 surgical and autopsy specimens of the tumor core and the invading area. Higher numbers of IDH1-R132H-positive cells in the invading area correlated with a higher tumor grade. Double staining for IDH1-R132H and Ki-67 demonstrated that most invading cells that expressed IDH1-R132H were not stained by the Ki-67 antibody, and the ratio of Ki-67-positive cells among IDH1-R132H-positive cells was significantly lower in the invasion area than in the tumor core in all grades of glioma. These data suggest that higher grade gliomas have a greater invasive potential and that invading cells possess low proliferative capacity.

Objective: We report a case of transbrachial right carotid artery stenting (CAS) without intra-aortic manipulation. Case presentation: A 71-year-old man presented with right visual field disturbance due to occlusion of the branch retinal artery and symptomatic right internal carotid artery stenosis. Because he had undergone aortic arch replacement for an aortic aneurysm, we planned CAS using the transbrachial approach without intra-aortic manipulation. During the guiding-sheath cannulation, it was difficult to insert a diagnostic catheter directly from the right subclavian artery to the right common carotid artery owing to the acute angle of these arteries. Using the pigtail catheter, the guidewire was easily inserted into the external carotid artery, and stenting was performed successfully. Conclusion: The pigtail catheter is an useful device for cannulating the guidewire through an acute-angled bifurcation. 【目的】経上腕動脈アプローチによる右頚動脈ステント留置術の際に右鎖骨下動脈（subclavian artery; SA）と総頚動脈（common carotid artery; CCA）の角度が強いと直接の誘導が難しい．この状況でCCA にガイドワイヤを容易に挿入する方法を紹介する．【症例】71 歳男性．黒内障で発見された右内頚動脈狭窄症で紹介された．胸部大動脈瘤で弓部置換術後であったため経上腕アプローチで大動脈内操作を行わないステント留置術を計画した．右SA と右CCA の角度が強く直接ガイドワイヤを進められなかったが，ピッグテールカテーテルの使用により容易に誘導できステント留置術を施行し得た．【結論】上腕動脈経由の右CCA への直接誘導においてピッグテールカテーテルは有用であった．

We report a rare case of a hemodialysis patient with a calcified amorphous tumor (CAT) on both sides of the atrioventricular valve annulus. A 70-year-old female who had received hemodialysis for 23 years because of chronic glomerulonephritis presented to our hospital with acute heart failure. Echocardiography indicated the presence of mobile cardiac masses on the mitral valve and tricuspid valve annulus. We suspected the presence of a cardiac tumor or vegetation. The patient received 3 g/day sulbactam-ampicillin and 60 mg/day gentamicin. Surgery was performed on the 14th day after hospital admission. The patient underwent mitral valve replacement, tricuspid annuloplasty, and tumor resection. Based on the pathological findings, the cardiac tumor was diagnosed as a CAT.

We treated a case of recurrent glioblastoma (GBM) with bevacizumab and assessed its effect biologically. A 55-year-old man with a left frontal lobe GBM was experiencing recurrence 7 months postoperation. We administered bevacizumab concomitant with temozolomide (TMZ). Follow-up magnetic resonance imaging (MRI) showed dramatic but temporal tumor reduction; however, the patient died of re-recurrent disease 6 months after beginning bevacizumab. We obtained an autopsy and analyzed the detailed molecular change. In the autopsy specimen, the quantity of microvessels was significantly reduced. Vascular endothelial growth factor receptor (VEGFR) 1 and VEGFR2 were downregulated, most likely due to a negative feedback mechanism by blocking of VEGF signaling. Matrix metalloproteinase (MMP)-2 and membrane-type 1 MMP were upregulated, resulting in the higher activation of MMP-2 in the autopsy specimen. MIB-1 staining index and phosphorylation levels of p44/42-mitogen-activated protein kinase did not change, whereas phosphorylated protein kinase B (Akt) was decreased in the autopsy specimen, suggesting compensation and/or amplification of other proliferative signaling pathways such as suppression of apoptosis signaling. Consequently, bevacizumab might inhibit the VEGF autocrine loop, which then causes a change in molecular expression related not only to enhancement of tumor invasion but also maintenance of tumor proliferation.

Arteriovenous shunting visualized by angiography is one of the major features of glioblastomas, and the visualization is dependent on the presence of extensive shunting. Extensive arteriovenous shunting is associated with the risk of poorly controlled intraoperative bleeding. When a tumor with extensive arteriovenous shunting is located in close proximity to the eloquent regions of the brain, a meticulous surgical procedure is necessary. In the present study, the site-oriented visualization of angiographical arteriovenous shunting was evaluated from the perspective of surgical treatment, with a particular focus on the perisylvian region that is in close proximity to motor and language regions (dominant hemisphere), as well as large arteries and veins. Twenty-six consecutive patients underwent a resection of glioblastoma between February 2007 and September 2012. All patients were presurgically examined using digital subtraction angiography. The patients were subdivided into the following two groups based on the location of the tumor: 1) perisylvian glioblastoma (18 patients) and 2) non-perisylvian glioblastoma (eight patients). Angiography to detect the arteriovenous shunting was performed. In addition, the number of intratumoral vessels, tumor proliferative activity (MIB-1 labeling index), and volume of intraoperative bleeding were evaluated and compared between the two groups. Angiographical arteriovenous shunting was definitively visualized in 13 of 18 (72 %) perisylvian glioblastomas, in contrast to only one of eight (13 %) non-perisylvian glioblastomas (p = 0.007). There were no significant differences between the two groups with respect to the number of intratumoral vessels, MIB-1 labeling index, and volume of intraoperative bleeding. However, massive intraoperative bleeding of > 2,000 mL occurred in one perisylvian glioblastoma patient. Glioblastomas in the perisylvian region tend to be associated with extensive arteriovenous shunting that can be definitively visualized by performing an angiography. Because arteriovenous shunting carries the risk of intraoperative bleeding, perisylvian glioblastomas—particularly in the dominant hemisphere—should be resected with a meticulous surgical procedure and strategy.

Nonenhancing intrinsic brain tumors have been empirically treated with a strategy that has been adopted for World Health Organization (WHO) grade II gliomas (low-grade gliomas: LGGs), even though small parts of the tumors might have been diagnosed as WHO grade III gliomas after surgery. However, the best surgical strategy for nonenhancing gliomas, including LGGs, is still debatable. LGGs have the following features: slow growth, high possibility of histologically malignant transformation, and no clear border between the tumor and adjacent normal brain. We retrospectively examined 26 consecutive patients with nonenhancing gliomas who were surgically treated at Kanazawa University Hospital between January 2006 and May 2012, with special reference to functional reorganization, extent of resection (EOR), and functional mapping during awake surgery. These categories are closely related with the features of LGG, i.e. functional reorganization due to slow-growing nature, EOR with related malignant transformation, and functional mapping for delineating the unclear tumor border. Finally, we discuss surgical strategies for slow-growing gliomas that are represented by LGGs and nonenhancing gliomas. In conclusion, slow-growing gliomas tend to undergo functional reorganization, and the functional reorganization affects the presurgical evaluation for resectability based on tumor location related to eloquence. In the clinical setting, to definitely identify the reorganized functional regions, awake surgery is recommended. Therefore, awake surgery could increase the extent of the resection of the tumor without deficits, resulting in the delay of malignant transformation and increase in overall survival.

Background In photodynamic diagnosis using 5-aminolevulinic acid (5-ALA), discrimination between the tumor and normal tissue is very important for a precise resection. However, it is difficult to distinguish between infiltrating tumor and normal regions in the boundary area. In this study, fluorescent intensity and bright spot analyses using a confocal microscope is proposed for the precise discrimination between infiltrating tumor and normal regions. Methods From the 5-ALA-resected brain tumor tissue, the red fluorescent and marginal regions were sliced for observation under a confocal microscope. Hematoxylin and eosin (H&E) staining were performed on serial slices of the same tissue. According to the pathological inspection of the H&E slides, the tumor and infiltrating and normal regions on confocal microscopy images were investigated. From the fluorescent intensity of the image pixels, a histogram of pixel number with the same fluorescent intensity was obtained. The fluorescent bright spot sizes and total number were compared between the marginal and normal regions. Results The fluorescence intensity distribution and average intensity in the tumor were different from those in the normal region. The probability of a difference from the dark enhanced the difference between the tumor and the normal region. The bright spot size and number in the infiltrating tumor were different from those in the normal region. Conclusions Fluorescence intensity analysis is useful to distinguish a tumor region, and a bright spot analysis is useful to distinguish between infiltrating tumor and normal regions. These methods will be important for the precise resection or photodynamic therapy of brain tumors. © 2016 Elsevier B.V., Embargo Period 12 months

// Takuya Furuta 1 , Hemragul Sabit 1 , Yu Dong 1 , Katsuyoshi Miyashita 1 , Masashi Kinoshita 1 , Naoyuki Uchiyama 1 , Yasuhiko Hayashi 1 , Yutaka Hayashi 1 , Toshinari Minamoto 2 , Mitsutoshi Nakada 1 1 Department of Neurosurgery, Division of Neuroscience, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan 2 Division of Translational and Clinical Oncology, Cancer Research Institute, Kanazawa University, Kanazawa, Japan Correspondence to: Mitsutoshi Nakada, email: mnakada@med.kanazawa-u.ac.jp Keywords: glioblastoma, GSK3β, drug repositioning, translational research, clinical study Received: May 11, 2016 Accepted: January 25, 2017 Published: February 09, 2017 ABSTRACT Background: Glycogen synthase kinase (GSK)-3β has emerged as an appealing therapeutic target for glioblastoma (GBM). Here, we investigated the therapeutic effect of the current approved drugs against GBM via inhibition of GSK3β activity both, in experimental setting and in a clinical study for recurrent GBM patients by repositioning existent drugs in combination with temozolomide (TMZ). Materials and Methods: Progression-free and overall survival rates were compared between patients with low or high expression of active GSK3β in the primary tumor. GBM cells and a mouse model were examined for the effects of GSK3β-inhibitory drugs, cimetidine, lithium, olanzapine, and valproate. The safety and efficacy of the cocktail of these drugs (CLOVA cocktail) in combination with TMZ were tested in the mouse model and in a clinical study for recurrent GBM patients. Results: Activation of GSK3β in the tumor inversely correlated with patient survival as an independent prognostic factor. CLOVA cocktail significantly inhibited cell invasion and proliferation. The patients treated with CLOVA cocktail in combination with TMZ showed increased survival compared to the control group treated with TMZ alone. Conclusions: Repositioning of the GSK3β-inhibitory drugs improved the prognosis of refractory GBM patients with active GSK3β in tumors. Combination of CLOVA cocktail and TMZ is a promising approach for recurrent GBM.

【目的】左総頚動脈（CCA）狭窄症に対するステント留置術の際にガイディングシースの保持を右上腕動脈経由のGooseNeck Snareで行った症例を報告する．【症例】70歳，男性．一過性の右片麻痺で発見された症候性左CCA狭窄症に対してステント留置術を行った．狭窄病変とCCA起始部の距離が短いためガイディングシースを十分遠位まで挿入できなかった．そのため右上腕動脈経由のGooseNeck Snareでガイディングシースを保持してステント留置術を施行し得た．【結論】上腕動脈経由のGooseNeck Snareによる左CCAのガイディングシースの保持は有用であった．Objective: We report a case of left common carotid artery stenting in which the guide sheath was held in place by a GooseNeck Snare inserted from the right brachial artery. Case presentation: A 70-year-old man presented with transient right hemiparesis and symptomatic left common carotid artery stenosis. During carotid artery stenting, we were unable to insert the guide sheath into the distal site of the carotid artery because of the short distance between the origin of the carotid artery and the stenotic lesion. By holding the guide sheath in place using the GooseNeck Snare inserted from the right brachial artery, stenting was performed successfully. Conclusion: Transbrachial GooseNeck Snare was useful for stabilization of the guide sheath during left common carotid artery stenting.

Schwannomas of the orbit are very rare benign neoplasms. Intraorbital cystic schwannomas originating from the frontal nerve are even rarer, with only 1 case reported to date. This is most likely due to the fact that, in most cases, the origin of the orbital schwannoma cannot be identified intraoperatively. The nerve origin is usually speculated from histological examination of the specimen and the postoperative neurological deficits of the patient. Here, we present the case of a 65-year-old woman with a one-month history of exophthalmos, whose orbital cystic lesion was completely removed by microsurgical transcranial operation. Intraoperatively, the continuity between the tumor and frontal nerve was seen macroscopically, leading us to confirm the frontal nerve as an origin of the tumor, which was consistent with the postoperative neurological findings. The diagnosis of the tumor was established as schwannoma from the histological examination. As a differential diagnosis of the orbital cystic lesions, the possibility of schwannomas should be kept in mind.

Background and importance We report the case of a patient with multiple neuroepithelial tumors of different histological cell types. In this patient, synchronous appearance of tumors of the central nervous system, ie, cervical ependymoma and a fourth ventricle choroid plexus papilloma, was noted. Clinical presentation A 36-year-old man presented with sensory disturbance of the bilateral upper extremities. Magnetic resonance imaging revealed a heterogeneously enhanced mass with syringomyelia in the upper cervical cord and a well-enhanced mass in the fourth ventricle. The patient underwent bilateral suboccipital craniotomy and laminectomy from the level of C1 to C3 for both lesions. The lesions were grossly resected. Histological and immunohistochemical examinations revealed that the cervical tumor was an ependymoma, and the fourth ventricle tumor was a choroid plexus papilloma. Conclusion The appearance of synchronous multiple neuroepithelial tumors of different histological cell types is extremely rare, and this is the first known case of ependymoma occurring synchronously with choroid plexus papilloma, both tumors having different histological cell types. Displacement and maldifferentiation of primitive, multipotent neuroepithelial cells may be a causative factor for this unique presentation.

Choroid plexus carcinoma (CPC) is a malignant tumor with a strong tendency to spread along the cerebrospinal fluid pathway. There is no standardized chemotherapy protocol for this rare tumor. We report a 38-year-old man with CPC in the lateral ventricle with obstructive hydrocephalus. Because of the poor demarcation between thalamus and fornix, subtotal tumor resection was performed. Postoperative spine magnetic resonance (MR) image revealed whole spinal axis dissemination. After diagnosis of CPC, the patient was treated with whole ventricular and spine radiation concomitant with temozolomide chemotherapy, although the O 6-methylguanine-DNA methyltransferase (MGMT) promoter was found to be unmethylated. Although MR images revealed transient stable disease during adjuvant therapy, tumor progression was depicted after four cycles of temozolomide therapy. We discuss the ineffectiveness of adjuvant temozolomide therapy for CPC in connection with O 6-methylguanine-DNA methyltransferase promoter methylation. © 2011 The Japan Society of Brain Tumor Pathology.

Few studies have assessed physical and psychological status in long-term survivors of childhood solid tumors in Japan. For children with such diseases diagnosed and treated in our hospital, our purpose was to clarify the physical and psychological status of long-term survivors and their parents. Subjects were 56 patients who were diagnosed at our institution as having a childhood malignant solid tumor between 1982 and 2005 and had been alive for at least 5 years after treatment. Surveys were sent and returned by mail. Of the 56 patients surveyed, 32 responded. The current health condition and psychosocial status of survivors were evaluated as good by their parents. However, psychological tests revealed psychosocial problems in 28.1% of the children. Severe posttraumatic stress associated with the child’s disease and its treatment was present in 15.6% of the parents. Physical status of long-term survivors of childhood malignant solid tumors was good in general. However, psychological tests revealed psychosocial problems in some of the children and posttraumatic stress in the parents. Considering the diversity of both the diseases and their clinical course, a qualitative study is warranted for further analysis.

Background: Recurrence of glioma frequently occurs within the marginal area of the surgical cavity due to invading residual cells. 5-Aminolevulinic acid (5-ALA) fluorescence-guided resection has been used as effective therapeutic modalities to improve discrimination of brain tumour margins and patient prognosis. However, the marginal areas of glioma usually show vague fluorescence, which makes tumour identification difficult, and the applicability of 5-ALA-based photodynamic therapy (PDT) is hampered by insufficient therapeutic efficacy in glioma tissues. Methods: To overcome these issues, we assessed the expression of ferrochelatase (FECH) gene, which encodes a key enzyme that catalyses the conversion of protoporphyrin IX (PpIX) to heme, in glioma surgical specimens and manipulated FECH in human glioma cell lines. Results: Prominent downregulation of FECH mRNA expression was found in glioblastoma tissues compared with normal brain tissues, suggesting that FECH is responsible for PpIX accumulation in glioblastoma cells. Depletion of FECH by small interference RNA enhanced PpIX fluorescence after exposure to 5-ALA concomitant with increased intracellular PpIX accumulation in glioma cells. Silencing of FECH caused marked growth inhibition and apoptosis induction by PDT in glioma cells. Conclusion: These results suggest that knockdown of FECH is a potential approach to enhance PpIX fluorescent quality for optimising the subjective discrimination of vague fluorescence and improving the effect of 5-ALA-PDT.

We evaluated the radiologic and clinical outcome of unruptured intracranial aneurysms treatment by using double microcatheters into the aneurysm. Between October 2006 and December 2009, 30 patients with 30 aneurysms were treated, including 12 patients treated by using double microcatheters and 18 patients treated by using a single microcatheter. The volume embolization ratio (VER) ―percentage of the coil volume occupying the aneurysmal lumen―was used as an index to evaluate the radiologic results. The VER of aneurysms treated with double-microcatheter technique was 25.2±4.8%, and the VER of aneurysms treated with the single-microcatheter technique was 21.6±3.9% (PAlthough in the aneurysms with a long aneurysmal diameter/short aneurysmal diameter (L/S) ratio less than 1.5, there were no significant differences between the 2 groups, in the aneurysms with a L/S ratio of 1.5 or greater, the VER of aneurysms treated with the double-microcatheter technique was 27.6±4.3%, and the VER of aneurysms treated with single-catheter technique was 19.7±3.4% (PThe double microcatheter technique is an effective and safe treatment for unruptured aneurysms.

We experienced an unusual case of a 15-year-old girl who suffered acute bilateral blindness caused by a frontal lobe tumour. She underwent emergent operation, after which her vision recovered. This case emphasizes that a brain mass can cause sudden onset blindness and an emergency intervention might save the patient's sight.

A retro-odontoid pseudotumor (ROP) is commonly associated with atlantoaxial subluxation (AAS). Here, we report a patient with ROP but without AAS. The patient was a 72-year-old man who did not have a history of rheumatoid arthritis or trauma to the head and neck. The patient was admitted to our hospital with gait disturbance, progressive motor weakness in both upper extremities and sensory disturbance in all four extremities. MRI showed a retro-odontoid mass with severe compression of the cervical spinal cord. A CT scan showed spondylotic changes in C5, C6, and C7 and bilateral facet fusion between C3 and C4. Dynamic radiography showed no evidence of AAS; there was loss of mobility at C2-C7 and excessive mobility at C1. Intraoperative pathological examination revealed that the lesion was a pseudotumor; therefore, posterior C1-C2 fixation was performed. MRI performed 6 months after the operation revealed that the pseudotumor was markedly reduced. To the best of our knowledge, patients with ROP without AAS are uncommon.

Background Langerhans cell histiocytosis (LCH) is a rare disease that may affect the central nervous system; it is caused by dendritic cell proliferation, and typically occurs in children. LCH frequently appears in the pituitary stalk and rarely results in multiple enhanced lesions in the brain parenchyma. Case description We present a case of a 40-year-old woman who deveolped panhypopituitarism and central diabetes insipidus in the postpartum period requiring hormone replacement therapy. At first, magnetic resonance imaging only revealed thickening of the pituitary stalk; while 6 months later, a single enhanced mass lesion was detected in the hypothalamus. Another 5 months later, the lesion had enlarged with appearance of multiple, enhanced satellite lesions in the basal ganglia and white matter. The patient underwent successful craniotomy to obtain a biopsy sample; LCH of the hypothalamus was definitively diagnosis by histopathological examination. Steroids were administrated and resulted in significant reduction of all lesions. Conclusions Definitive histopathological diagnosis and subsequent appropriate therapy, such as steroid administration, are required when LCH lesions in the hypothalamus become progressively enlarged and new lesions appear in the brain parenchyma.

Four patients presented with intracerebral hemorrhage secondary to ventriculoperitoneal (VP) shunt insertion. VP shunt insertion was performed for idiopathic normal-pressure hydrocephalus (Case 1), hydrocephalus after cerebellar hemorrhage (Case 2), and subarachnoid hemorrhage followed by meningitis (Cases 3 and 4). Cerebral hemorrhage was confirmed 4 hours (Case 1), 2 days (Case 2), 7 days (Case 3), and 13 days (Case 4) after the operation. Cases 1 and 2 required intraoperative hemostasis for bleeding from the cortical vein. The 7 previous and our 4 patients were divided according to early (within 2 days after shunt placement, n = 6, Group 1) and delayed (5-13 days post-shunting, n = 5, Group 2) hemorrhage. Bleeding was attributable to venous occlusion due to intraoperative manipulation in Group 1, and to the vulnerability of brain tissue induced by a primary brain disease in Group 2.

A 69-year-old woman developed hydrocephalus after the embolization of an incidentally detected unruptured large internal carotid artery aneurysm with bare platinum coils. Endovascular embolization resulted in near-total aneurysm occlusion. She complained of mild headache 18 hours after the procedure and magnetic resonance (MR) imaging performed on the 6th post-treatment day revealed wall enhancement and perianeurysmal brain edema. Follow-up MR imaging showed recanalization of the aneurysm and gradual ventricular enlargement. She presented with typical symptoms of hydrocephalus including disorientation, gait disturbance, and urine incontinence at 7 months post-embolization. We removed 30 ml of cerebrospinal fluid by lumbar tap, which improved her condition without symptom recurrence for 2 months. She did not require shunt placement. Post-interventional hydrocephalus is known in patients with unruptured aneurysms embolized with bioactive second-generation coils. This case shows that hydrocephalus can occur after aneurysm embolization with bare platinum coils without intracranial hemorrhage.

緩和ケア領域で経験する頭頸部がんや各種がんの皮膚転移, 非切除乳がんなどの体表部悪性腫瘍の出血に対しては, 有用な方法がなく止血に難渋していた. 今回われわれは, 中咽頭がん再発病巣から出血を繰り返し, 1日に5回前後の包交を必要としていた患者に対して, Mohsペーストを使用することにより著明な止血効果と滲出液やにおいの軽減が認められた症例を経験したので報告する. Mohsペーストは, 安価な材料を用いて院内調製が可能であり, その作用機序は主成分の塩化亜鉛が潰瘍面の水分によりイオン化し, 亜鉛イオンのタンパク凝集作用によって腫瘍細胞や腫瘍血管, および二次感染した細菌の細胞膜が硬化することによる. 本症例においてMohsペーストは, 予後の限られた患者が出血や滲出液, においに悩まされることなくQOLを維持するために効果的であった. 今後, 製剤の安定性や使用方法が確立され, 本法が普及することが期待される. Palliat Care Res 2009; 4(2): 346-350

Both the dentate line (DL) and anorectal line (ARL) are anatomic landmarks for rectal mucosal dissection during transanal pull-through for Hirschsprung disease. We compared outcome after rectal mucosal dissection commencing above the DL (DL group; n = 8) with outcome after rectal mucosal dissection commencing on the ARL (ARL group; n = 6) with normal babies (Cont group; n = 10).Rectal mucosal dissection commenced on the ARL in the ARL group and at various levels (0-10 mm) above the DL in the DL group. Outcome was assessed prospectively for 6 months using a standard structured questionnaire.Subject demographics were not significantly different. Differences in frequency of motions between the ARL and Cont groups were not statistically significant after 3 months of age, but the DL group had significantly more motions than the other 2 groups after 4 months of age (P.01). At 6 months of age, fecal staining was 17% in the ARL group, 63% in the DL group, and 0% in the Cont group. Anal shape was normal in the ARL and Cont groups, but 50% of the DL group had visible anal mucosa.Bowel function in the ARL group is similar to normal, and because the ARL is easily identifiable without the need for subjective interpretation, we recommend using the ARL as a landmark for rectal mucosal dissection during transanal pull-through for Hirschsprung disease.

OBJECTIVE We report our experience of shunt infections (SIs) following intestinal cystoplasty (CP) in patients with neurogenic bladder (NB) and hydrocephalus. METHODS We reviewed 34 patients with NB who underwent intestinal CP between 1984 and 2005. All had ventriculoperitoneal (VP) shunts converted to ventriculopleural (VL) shunts prior to intestinal CP to prevent SIs. SI secondary to intestinal CP was defined as infection within 30 days of intestinal CP. RESULTS No SIs were reported. Mean age at shunt conversion was 9.5 years (range, 2–25 years), mean length of follow-up after conversion was 12.8 years (range, 2–18 years), and mean period between conversion and CP was 12 days (range, 0–30 days). Pleural effusion (PE) occurred post-conversion in 13/34 subjects (41%). Ten were treated with diuretics for a mean of 12 days, two were treated conservatively, and one required revision to a ventriculoatrial shunt. Delayed PE occurred in two patients at 7 and 18 months, secondary to respiratory infections. In the literature, there are six reports of SIs after intestinal CP in 216 NB patients. Overall, seven of 216 patients (3.2%) had SI. CONCLUSION VP shunts should be converted to VL shunts prior to intestinal CP because this appears to prevent SIs.

In Japan, a nationwide programme between 1984 and 2003 screened all infants for urinary catecholamine metabolites as a marker for neuroblastoma. Before 1989, this was done by qualitative spot tests for vanillylmandelic acid in urine, and subsequently by quantitative assay with high-performance liquid chromatography (HPLC). However, the Japanese government stopped the mass-screening programme in 2003, after reports that it did not reduce mortality due to neuroblastoma. We aimed to assess the effectiveness of the programme, by comparing the rates of incidence and mortality from neuroblastomas diagnosed before 6 years of age in three cohorts.We did a retrospective population-based cohort study on all children born between 1980 and 1998, except for a 2-year period from 1984. We divided these 22,289,695 children into three cohorts: children born before screening in 1980-83 (n=6,130,423); those born during qualitative screening in 1986-89 (n=5,290,412); and those born during quantitative screening 1990-98 (n=10,868,860). We used databases from hospitals, screening centres, and national cancer registries. Cases of neuroblastoma were followed up for a mean of 78.7 months.21.56 cases of neuroblastoma per 100,000 births over 72 months were identified in the qualitatively screened group (relative risk [RR] 1.87, 95% CI 1.66-2.10), and 29.80 cases per 100,000 births over 72 months in the quantitatively screened group (RR 2.58, 2.33-2.86). The cumulative incidence of neuroblastoma in the prescreening cohort (11.56 cases per 100,000 births over 72 months) was lower than that in other cohorts (p0.0001 for all comparisons), but more neuroblastomas were diagnosed after 24 months of age in this cohort (p=0.0002 for qualitative screening vs prescreening, p0.0001 for quantitative screening vs prescreening). Cumulative mortality was lower in the qualitative screening (3.90 cases per 100,000 livebirths over 72 months) and quantitative screening cohorts (2.83 cases) than in the prescreening cohort (5.38 cases). Compared with the prescreening cohort, the relative risk of mortality was 0.73 (95% CI 0.58-0.90) for qualitative screening, and 0.53 (0.42-0.63) for quantitative screening. Mortality rates for both the qualitative and quantitative screening groups were lower than were those for the prescreening cohort (p=0.0041 for prescreening vs qualitative screening, p0.0001 for prescreening vs quantitative screening).More infantile neuroblastomas were recorded in children who were screened for neuroblastoma at 6 months of age than in those who were not. The mortality rate from neuroblastoma in children who were screened at 6 months was lower than that in the prescreening cohort, especially in children screened by quantitative HPLC. Any new screening programme should aim to decrease mortality, but also to minimise overdiagnosis of tumours with favourable prognoses (eg, by screening children at 18 months).

The objective of this study is to analyze the outcome of surgery for high/intermediate-type imperforate anus using anal endosonography (AES), magnetic resonance imaging (MRI), and a continence evaluation questionnaire (CEQ).In this study, 24 cases of high/intermediate-type imperforate anus were studied. Fifteen of 24 had Georgeson's laparoscopy-assisted anorectoplasty (GLA), and 9 of 24 had Pena's posterior sagittal anorectoplasty (PPA). All subjects had AES and MRI postoperatively. On AES, differences in thickness of the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock were compared, and if pull-through colon was central, AES was 0. On MRI, differences in thickness were analyzed semiquantitatively and scored; if muscles were of even thickness, the score was 0; slight difference was 1, and marked difference was 2. A 5-parameter CEQ questionnaire (maximum score, 10) was administered to 16 of 24 subjects followed up for more than 3 years (9 GLA, 7 PPA). Surgical stress was assessed using mean febrile period, duration of raised white blood cell count, and peak C-reactive protein level.Mean age at surgery and mean postoperative period for both groups were not statistically different. There were no differences in mean muscle thickness for ES or PR on AES according to procedure (ES: GLA = 0.19 +/- 0.15 mm, PPA = 0.16 +/- 0.09 mm, P = .59; PR: GLA = 0.19 +/- 0.19 mm, PPA = 0.22 +/- 0.15 mm, P = .69). Magnetic resonance imaging scores were also not significantly different according to procedure (GLA: 0.77 +/- 0.83; PPA: 0.75 +/- 0.50, P = .97). When CEQ were compared annually, scores for GLA were generally higher throughout the study but only statistically significant at 3 and 4 years (P.05). Differences in parameters of surgical stress were not significant.Although there were no differences in muscle thickness and parameters of surgical stress observed according to technique, GLA would appear to provide better outcome based on CEQ scores.

Our purpose was to clarify factors that influence the level of depression of Japanese children with anorectal malformations (ARM). The subjects comprised 66 children with ARM, aged 0-16 years, and their mothers. Patients were divided into three groups (Group 1: aged 0-5 years, Group 2: 6-11 years, and Group 3: 12-16 years). The level of depression of the children aged 6-16 years was investigated by Kovac's children's depression inventory (CDI). The psychological status of their mothers was assessed by Spielberger's state-trait anxiety index (STAI) and Zung's self-rating depression scale (SDS). The classification of ARM and the clinical condition were also investigated. Defecation scores were assigned for the degree of bowel dysfunction. The level of depression in the patients was more marked in Group 3 than in Group 2 according to the CDI score. Significant correlations between the CDI score of the child and the STAI-1, STAI-2 and SDS scores of the mother were observed in Group 2, but not in Group 3. In Group 2, the STAI-1 score of the mothers was significantly influenced by the degree of bowel dysfunction in her child. In Group 3, the CDI score was significantly correlated with the constipation score. This study revealed that bowel dysfunction is the important factor that influences the level of depression of the children with ARM. In the period of childhood during which the child attends primary school, bowel dysfunction indirectly influences the level of depression of children through the psychological status of mothers. However, bowel dysfunction directly influences the level of depression of children in adolescent patients.

Neuroblastoma is a heterogeneous tumor and that may have a favorable or unfavorable prognosis. In Japan, a nation-wide neuroblastoma mass-screening (MS) project assessed 6-month-old infants between 1985 and 2003, and almost all neuroblastomas, including regressing or maturing tumors were thought to be detected in this period. To evaluate the heterogeneity of neuroblastoma subgroups, we analyzed patients with neuroblastoma who had been diagnosed during this period. The clinical courses of 4,209 patients with neuroblastoma, including 1,560 MS detected patients, whose tumors had been diagnosed between 1971 and 1995 were registered. The 2,520 cases registered between 1985 and 1995 were compared to 1,050 cases registered between 1971 and 1980 and analyzed by a multi-gene target model to determine the age distribution of neuroblastoma incidence. We hypothesized that three target genes were responsible for the progression of neuroblastoma: one pair of tumor suppressor gene alleles, one oncogene, and one gene controlling regression/differentiation. This simulation study revealed that the age distribution at initial diagnosis of neuroblastoma was divided into four groups based on post-fertilization age: 20-40, 40-50, 60-90, and 160-200 weeks. Since neuroblatoma in the first group occurred prenatal, post-natal clinical neuroblastoma can be classified into three age groups: 0-6 months, 1-2 years, and 3-4 years. The 0- to 6-month group consisted of mostly benign tumors, and the two older groups had predominantly malignant phenotypes. Our proposed model could explain qualitatively the distribution of neuroblastoma consisting of one subgroup with a favorable prognosis and two subgroups with unfavorable prognosis. For clinically relevant risk stratification, an age cutoff should be considered by the age distribution of these heterogeneous subgroups.

To prepare for a Japanese nationwide group study of patients with rhabdomyosarcoma (RMS), we examined the characteristics and outcomes of RMS patients treated recently in Japan.We classified 331 RMS patients treated between 1991 and 2002 at 63 institutions according to the Intergroup Rhabdomyosarcoma Study V (IRS-V) risk-group classification.Ten-year survival rates were 86.3% for patients in low-risk subgroup A, 80.7% for low-risk subgroup B, 62.7% for intermediate-risk subgroup A, 61.7% for intermediate-risk subgroup B, and 38.1% for the high-risk group. The outcomes of the patients in the former three groups were 8%, 12%, and 21% worse than the outcomes of the respective patients in the IRS-III and early IRS-IV data. The frequency of the alveolar histological subtype was 21.8%. Chimera genes, which are useful markers for the alveolar subtype, had been examined in only 10% of the patients treated in the period of this investigation. The survival rates of our patients with embryonal and alveolar histological subtypes (65.9% and 63.4%, respectively) were not significantly different. Among the patients in the high-risk group, the 5-year survival of patients who received high-dose chemotherapy (HDC; 58.2%) was significantly better than that of patients who did not receive HDC (18.4%).Patients in the lower-risk groups with embryonal-type tumors had poorer outcomes in this retrospective study. The better outcome of patients in the high-risk group is apparently due to the outstanding results obtained with an HDC regimen in a single institution. These results suggest that there is a need for: (1) a standard therapy, (2) a rapid central pathology review including a chimera gene analysis for the lower-risk group, and (3) evaluation of the efficacy of the high-dose regimen for the high-risk group in Japan.

Awake surgery could be a useful modality for lesions locating in close proximity to the eloquent areas including primary motor cortex and pyramidal tract. In case with supplementary motor area (SMA) lesion, we often encounter with intraoperative motor symptoms during awake surgery even in area without positive mapping. Although the usual recovery of the SMA syndrome has been well documented, rare cases with permanent deficits could be encountered in the clinical setting. It has been difficult to evaluate during surgery whether the intraoperative motor symptoms lead to postoperative permanent deficits. The purpose of this study was to demonstrate the intraoperative motor symptoms could be reversible, further to provide useful information for making decision to continue surgical procedure of tumor resection. Eight consecutive patients (from July 2012 to June 2014, six men and two women, aged 33-63 years) with neoplastic lesions around the SMA underwent an awake surgery. Using a retrospective analysis of intraoperative video records, intraoperative motor symptoms during tumor resection were investigated. In continuous functional monitoring during resection of SMA tumor under awake conditions, the following motor symptoms were observed during resection of the region without positive mapping: delayed motor weakness, delay of movement initiation, slowness of movement, difficulty in dual task response, and coordination disturbance. In seven patients hemiparesis observed immediately after surgery recovered to preoperative level within 6 weeks. During awake surgery for SMA tumors, the above-mentioned motor symptoms could occur in area without positive mapping and might be predictors for reversible SMA syndrome.

Duodenal stenosis is a type of congenital intestinal atresia syndrome. Most patients are diagnosed during the neonatal period based on recurrent vomiting, but some cases develop symptoms at an older age. We report a case with an unusual pathogenic mechanism: sudden ileus due to food impaction.A 15-month-old boy was admitted to our institution with a history of recurrent vomiting and diarrhea for 7 days and fever for 2 days. Abdominal distention was found upon physical examination but other congenital anomalies were not. He was initially treated for acute gastroenteritis, but vomiting resumed after starting oral ingestion. Congenital membranous duodenal stenosis and impaction of a shimeji mushroom into the narrow section of the duodenum was recognized upon endoscopy. Dilatation was successful and restenosis was not seen. Food impaction is a rare (but important) initial event in congenital duodenal stenosis, and this disorder should be included in the differential diagnosis, especially for infants/toddlers with recurrent vomiting that develops at the time of eating solid and more diverse types of food.

Background/Purpose Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome. Methods A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994. Results Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years. Conclusions Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.

We reviewed the long-term outcome of sigmoidocolocystoplasty.The records of 86 patients who underwent sigmoidocolocystoplasty with a followup of more than 10 years were reviewed, focusing on histopathology, renal function, vesicoureteral reflux, bladder stones and urinary continence. We developed a urinary continence evaluation score, calculated as the postoperative minus the preoperative urinary continence score, including 0-incontinence, 1-severe leakage, 2-moderate leakage, 3-mild leakage, 4-almost dry and 5-dry, for patient self-assessment of urinary continence. A score of 2 or more meant improvement and a score of less than 2 meant no improvement.Mean followup was 13.1 years (range 10 to 21). Histopathology showed inflammation in all cases, hyperplasia in 9 and metaplasia in 4. In 80 of 86 cases creatinine clearance was normal. Ureteral reimplantation had been performed before sigmoidocolocystoplasty in 9 cases (13 kidneys) for grades III to V vesicoureteral reflux, which recurred as grade I in 1 (1 kidney). Ureteral reimplantation was performed during sigmoidocolocystoplasty in 31 cases (58 kidneys) and grades I to II vesicoureteral recurred in 5 (6 kidneys). Of the remaining 44 sigmoidocolocystoplasty cases there were 2 (2 kidneys) of grade I vesicoureteral reflux. On (99m)technetium-dimercapto-succinic acid scintigraphy there was increased renal scarring in 8 cases. Bladder stones were identified in 18 of 86 cases during followup but they were absent at review. The urinary continence evaluation score showed improvement in 61 cases and no improvement in 25.Our results suggest that sigmoidocolocystoplasty is safe and effective in the long term.

The aim of this study is to investigate the efficacy of dynamic computed tomography (CT) during selective angiography (CT-arteriography) of orbital tumors in the evaluation of intratumoral vascular anatomy, feeding artery territory, and histological diagnosis. Among 35 consecutive cases with various orbital lesions, those cases showing tumor staining or pooling of the contrast medium on digital subtraction angiography (DSA) were evaluated by CT-arteriography (n = 14). The information obtained by CT-arteriography was compared with that provided by enhanced MRI (n = 31) and dynamic MRI (n = 21), in which the contrast medium was injected intravenously. In addition to the visualization of fine vascular anatomy, CT-arteriography emphasized areas of nodular enhancement and non-enhancing cystic/necrotic components as well as the intratumoral feeding arteries. Patterns of CT-arteriography were categorized into three subgroups: homogeneous enhancement (benign lymphoid lesion), partial enhancement (schwannomas and carcinomas), and patchy multinodular enhancement (specific for cavernous angiomas). In addition, CT-arteriography with selective arterial catheterization clearly delineated the feeding artery territories. CT-arteriography, with a minimal dose of contrast medium, can offer significant advantages over intravenously injected dynamic neuroimaging, and provides additional valuable preoperative information about the orbital tumor under investigation.