The nonprogressive unilateral intracranial arteriopathy known as transient (focal) cerebral arteriopathy is not a well-recognized arteriopathy among practitioners of Korea and Japan, although it cannot be easily differentiated from early moyamoya disease. This review summarizes the nomenclature, pathophysiology, diagnostic evaluation, clinico-radiological features, and management of nonprogressive (reversible or stable) unilateral arteriopathy based on the relevant literature and our own experiences. Nonprogressive unilateral arteriopathy should be strongly suspected in children presenting with basal ganglia infarction and arterial beading. The early identification of patients likely to have nonprogressive or progressive arteriopathy would ensure proper management and guide further research for secondary stroke prevention.
Basal Ganglia ; Child* ; Humans ; Infarction ; Japan ; Korea ; Moyamoya Disease ; Stroke*

A case of congenital tectal lipoma found incidentally at an autopsy is reported. This female fetus was a product of pregnancy termination at 24 weeks of gestational age after sonographic detection of hydrocephalus. Autopsy revealed a small mass in the periaqueductal portion. The mass was composed of adipose tissue, cartilage, and mature bony tissue with hematopoiesis. The resultant diagnosis was tectal lipoma with osteocartilagenous element. It is uncertain whether the lesion represents a teratoma or hamartoma or mesenchymal metaplasia. The osteocartilagenous component suggests the latter.
Adult ; Autopsy ; Bone and Bones/pathology ; Brain Neoplasms/*congenital/pathology ; Cartilage/pathology ; Case Report ; *Corpora Quadrigemina/pathology ; Female ; Human ; Infant, Newborn ; Lipoma/*congenital/pathology ; Pregnancy

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

Cryopyrin-associated periodic syndrome (CAPS) is a hereditary autoinflammatory syndrome caused by mutations in NLRP3 (encoding cryopyrin), which presents with fever, fatigue and arthralgia. Thus far, however there have been no reports of CAPS in Korea. Herein, we report 3 cases of CAPS for the first time in Korea. The first case, a 28-year-old man with recurrent urticaria, arthralgia and fever induced by cold, all of which were observed in his father, showed elevated erythrocyte sedimentation rate and C-reactive protein. He exhibited a p.Gly303Asp variant of the NLPR3 gene. The second case, a 2-year-old girl who had recurrent urticaria, arthritis and oral and genital ulcers, was positive for HLA B51 and a p.Glu569Lys mutation in exon 3 of the NLRP3 gene. Administration of anakinra greatly improved her symptoms. The third case, a 4-year-old boy who presented with recurrent urticaria, arthralgia, and fever, exhibited a p.Val72Met mutation in exon 1 of the NLRP3 gene. Administration of tocilizumab improved all of his symptoms. This small case series suggests that clinicians consider CAPS and conduct genetic studies when arthralgia and fever are accompanied by urticaria in Korea.
Adult ; Arthralgia ; Arthritis ; Blood Sedimentation ; C-Reactive Protein ; Child, Preschool ; Cryopyrin-Associated Periodic Syndromes ; Exons ; Fathers ; Fatigue ; Female ; Fever ; Humans ; Interleukin 1 Receptor Antagonist Protein ; Korea ; Male ; Ulcer ; Urticaria

OBJECTIVE: To evaluate the efficacy of clinical use of a intrauterine Sengstaken-Blakemore tube (S-B tube) in postpartum hemorrhage not controlled with medication and conservative treatment. METHODS: Retrospective review was done in 18 women with insertion of S-B tube into intrauterine cavity who did not controlled with medication and conservative treatment for postpartum hemorrhage. After third stage of labor, women were treated with prophylactic intramuscular Methylergonovine 0.2 mg and intravenous infusions of oxytocin routinely. If the postpartum bleeding continued, Misoprostol 800 microgram (per rectal) and intravenous infusions of Dinoprost 2 mg mixed with 5% D/S 500 cc were employed. And then women were examined for retained placenta and laceration of birth canal. Where necessary, retained placenta was removed and lacerations were sutured. If the postpartum bleeding did not controlled despite all of procedures, we decided use of intrauterine S-B tube. RESULTS: The mean age of the patients was 30.9+/-4.4 (27-39) years, their mean body weights and parities were 67.8+/-8.2 (56.2-85.7) kg, 1.8+/-0.8 (1-4) and mean gestational age was 38.5+/-2.9 (37-42) weeks respectively. The mean time from delivery to insertion of S-B tube was 107.6+/-94.0 (24-360) minutes. The mean filled normal saline amount was 190.5+/-35.2 (120-230) cc. Of 18 who was inserted of S B tube, 15 cases needed not additional surgical therapy. So success rate was 83.3%. CONCLUSION: Insertion of intrauterine S-B tube appears as a simple and effective means of treating postpartum hemorrhage not controlled with medication and conservative treatment.
Body Weight ; Dinoprost ; Female ; Gestational Age ; Hemorrhage ; Humans ; Infusions, Intravenous ; Lacerations ; Methylergonovine ; Misoprostol ; Oxytocin ; Parturition ; Placenta, Retained ; Postpartum Hemorrhage* ; Postpartum Period* ; Retrospective Studies

We present an unusual case of peritoneal catheter migration following a ventriculoperitoneal shunt operation. A 7-month-old infant, who had suffered from intraventricular hemorrhage at birth, was shunted for progressive hydrocephalus. The peritoneal catheter, connected to an 'ultra small, low pressure valve system' (Strata(R); PS Medical,Gola, CA, USA) at the subgaleal space, was placed into the peritoneal cavity about 30 cm in length. The patient returned to our hospital due to scalp swelling 21 days after the surgery. Simple X-ray images revealed total upward migration and coiling of the peritoneal catheter around the valve. Possible mechanisms leading to proximal upward migration of a peritoneal catheter are discussed.
Catheters ; Hemorrhage ; Humans ; Hydrocephalus ; Infant ; Parturition ; Peritoneal Cavity ; Scalp ; Ventriculoperitoneal Shunt

OBJECTIVE: The purpose of this study was to investigate the clinical features and outcomes of pediatric cavernous malformation (CM) in the central nervous system. METHODS: Twenty-nine pediatric patients with supratentorial CM underwent microsurgical excision. In selected cases, transparent tubular retractor system (TTRS) was used to reduce retraction injury and intraoperative neuromonitoring (IONM) was held to preserve functioning cortex. Patients' demographics and symptoms were reviewed and surgical outcomes were discussed. RESULTS: The main initial clinical manifestations included the following : seizures (n=13, 45%), headache (n=7, 24%), focal neurological deficits (n=3, 10%), and an incidental finding (n=6, 21%). Overt hemorrhage was detected in 7 patients (24%). There were 19 children (66%) with a single CM and 10 (34%) children with multiple CMs. In 7 cases with deep-seated CM, we used a TTRS to minimize retraction. In 9 cases which location of CM was at eloquent area, IONM was taken during surgery. There was no major morbidity or mortality after surgery. In the 29 operated children, the overall long-term results were satisfactory : 25 (86%) patients had no signs or symptoms associated with CMs, 3 had controllable seizures, and 1 had mild weakness. CONCLUSION: With the assistance of neuronavigation systems, intraoperative neuromonitoring, and TTRS, CMs could be targeted more accurately and excised more safely. Based on the satisfactory seizure outcome achieved, complete microsurgical excision in children is recommended for CMs presenting with seizures but removal of hemosiderin-stained areas seems to be unnecessary.
Central Nervous System ; Child ; Demography ; Headache ; Hemangioma, Cavernous, Central Nervous System* ; Hemorrhage ; Humans ; Incidental Findings ; Mortality ; Neuronavigation ; Seizures

OBJECTIVES: The goal of this study was to define the radiologic characteristics of two-phase computed tomography (CT) of salivary gland Warthin tumors and to compare them to pleomorphic adenomas. We also aimed to provide a foundation for selecting a surgical method on the basis of radiologic findings. METHODS: We prospectively enrolled 116 patients with parotid gland tumors, who underwent two-phase CT preoperatively. Early and delayed phase scans were obtained, with scanning delays of 30 and 120 seconds, respectively. The attenuation changes and enhancement patterns were analyzed. In cases when the attenuation changes were decreased, we presumed Warthin tumor preoperatively and performed extracapsular dissection. When the attenuation changes were increased, superficial parotidectomy was performed on the parotid gland tumors. We analyzed the operation times, incision sizes, complications, and recurrence rates. RESULTS: Attenuation of Warthin tumors was decreased from early to delayed scans. The ratio of CT numbers in Warthin tumors was also significantly different from other tumors. Warthin tumors were diagnosed with a sensitivity of 96.1% and specificity of 97% using two-phase CT. The mean operation time was 38 minutes and the mean incision size was 36.2 mm for Warthin tumors. However, for the other parotid tumors, the average operation time was 122 minutes and the average incision size was 91.8 mm (P<0.05). CONCLUSION: Salivary Warthin tumor has a distinct pattern of contrast enhancement on two-phase CT, which can guide treatment decisions. The preoperative diagnosis of Warthin tumor made extracapsular dissection possible instead of superficial parotidectomy.
Adenolymphoma* ; Adenoma, Pleomorphic ; Diagnosis ; Humans ; Parotid Gland ; Parotid Neoplasms ; Prospective Studies ; Recurrence ; Salivary Glands* ; Sensitivity and Specificity ; Tomography, Spiral Computed*

The coexistence of moyamoya disease (MMD) with an arteriovenous malformation (AVM) is exceedingly rare. We report two cases of AVM associated with MMD. The first case was an incidental AVM diagnosed simultaneously with MMD. This AVM was managed expectantly after encephalo-duro-arterio-synangiosis (EDAS) as the main feeders stemmed from the internal carotid artery, which we believed would be obliterated with the progression of MMD. However, the AVM persisted with replacement of the internal carotid artery feeders by new external carotid artery feeders from the EDAS site. The AVM was eventually treated with gamma knife radiosurgery considering an increasing steal effect. The second case was a de novo AVM case. The patient was initially diagnosed with MMD, and acquired an AVM eight years later that was slowly fed by the reconstituted anterior cerebral artery. Because the patient remained asymptomatic, the AVM is currently being closely followed for more than 2 years without further surgical intervention. Possible differences in the pathogenesis and the radiologic presentation of these AVMs are discussed with a literature review. No solid consensus exists on the optimal treatment of MMD-associated AVMs. Gamma knife radiosurgery appears to be an effective treatment option for an incidental AVM. However, a de novo AVM may be managed expectantly considering the possible risks of damaging established collaterals, low flow characteristics, and probably low risks of rupture.
Anterior Cerebral Artery ; Arteriovenous Malformations ; Carotid Artery, External ; Carotid Artery, Internal ; Child ; Consensus ; Humans ; Intracranial Arteriovenous Malformations* ; Moyamoya Disease* ; Radiosurgery ; Rupture