OBJECTIVETo evaluate the diagnostic value and safety of flexible bronchoscopy in congenital great vessel diseases complicated with airway compression.

METHODThe medical records of patients with great vessels abnormalities who were admitted to the neonatal intensive care unit (NICU) from October 2005 to June 2009 were retrospectively reviewed; 34 cases were diagnosed as airway compression by flexible bronchoscopy, 10 cases as vascular ring, 24 cases as aortal arch obstruction. The age of the patients was 6 d - 11 m, body weight 2.2 - 8.7 kg [(4.6 +/- 1.4) kg]. Recorded airway abnormalities detected by bronchoscopy and CT, cardiac vascular defects and airway compression were consistent with the findings on operation. The relation between the airway compression and cardiac vascular abnormalities, treatment of the airway compression and outcome were analysed.

RESULTBronchoscopic assessment was successfully performed in NICU or operating room for all the patients. (1) Initial presentation of the 34 cases were tachypnea, stridor, refractory lung infection and prolonged mechanical ventilation. (2) Extrinsic compression was found in all the 10 cases with vascular ring by bronchoscopy initially which indicated vascular ring, airway compression was mainly of lower part of trachea. Diagnosis of 9 cases was consistent with CT diagnosis and in 1 case the diagnosis was confirmed by surgery; among these cases, 7 had congenital tracheal stenosis. (3) In the 24 cases with aortic obstructive lesion, 5 were detected to have tracheal stenosis by CT before correction of vascular abnormality, among whom one case was indicated to have tracheal stenosis by bronchoscopy, the other 19 cases were found with airway compression by bronchoscopy during or after vascular correction. Among the 24 cases, 21 had left main bronchial stenosis, 2 had congenital tracheal stenosis. Airway compression diagnosed by bronchoscopy agreed with the findings of CT. Two cases developed transient decrease of oxygen saturation, 5 cases developed transient tachycardia.

CONCLUSIONFlexible bronchoscopy plays an important role in assessment of the airway compression complicated with great vessel abnormalities. Bronchoscopy is an accurate, convenient, safe and rapid way for airway assessment, but further examination of the peripheral structure and vascular malformation need combined examination with CT.

Airway Obstruction ; diagnosis ; etiology ; Bronchoscopy ; methods ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Retrospective Studies ; Vascular Malformations ; complications ; diagnosis

Paragonimiasis is caused by ingesting crustaceans, which are the intermediate hosts of Paragonimus. The involvement of the brain was a common presentation in Korea decades ago, but it becomes much less frequent in domestic medical practices. We observed a rare case of cerebral paragonimiasis manifesting with intracerebral hemorrhage. A 10-yr-old girl presented with sudden-onset dysarthria, right facial palsy and clumsiness of the right hand. Brain imaging showed acute intracerebral hemorrhage in the left frontal area. An occult vascular malformation or small arteriovenous malformation compressed by the hematoma was initially suspected. The lesion progressed for over 2 months until a delayed surgery was undertaken. Pathologic examination was consistent with cerebral paragonimiasis. After chemotherapy with praziquantel, the patient was monitored without neurological deficits or seizure attacks for 6 months. This case alerts practicing clinicians to the domestic transmission of a forgotten parasitic disease due to environmental changes.
Animals ; Anthelmintics/therapeutic use ; Brain/parasitology/pathology ; Cerebral Hemorrhage/*etiology ; Child ; Dysarthria/etiology ; Facial Paralysis/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Paragonimiasis/*diagnosis/drug therapy/parasitology ; Paragonimus/isolation & purification ; Praziquantel/therapeutic use ; Tomography, X-Ray Computed ; Vascular Malformations/etiology

Paragonimiasis is caused by ingesting crustaceans, which are the intermediate hosts of Paragonimus. The involvement of the brain was a common presentation in Korea decades ago, but it becomes much less frequent in domestic medical practices. We observed a rare case of cerebral paragonimiasis manifesting with intracerebral hemorrhage. A 10-yr-old girl presented with sudden-onset dysarthria, right facial palsy and clumsiness of the right hand. Brain imaging showed acute intracerebral hemorrhage in the left frontal area. An occult vascular malformation or small arteriovenous malformation compressed by the hematoma was initially suspected. The lesion progressed for over 2 months until a delayed surgery was undertaken. Pathologic examination was consistent with cerebral paragonimiasis. After chemotherapy with praziquantel, the patient was monitored without neurological deficits or seizure attacks for 6 months. This case alerts practicing clinicians to the domestic transmission of a forgotten parasitic disease due to environmental changes.
Animals ; Anthelmintics/therapeutic use ; Brain/parasitology/pathology ; Cerebral Hemorrhage/*etiology ; Child ; Dysarthria/etiology ; Facial Paralysis/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Paragonimiasis/*diagnosis/drug therapy/parasitology ; Paragonimus/isolation & purification ; Praziquantel/therapeutic use ; Tomography, X-Ray Computed ; Vascular Malformations/etiology

Spinal dural arteriovenous fistula (SDAVF) is the most common spinal vascular malformation, however it is still rare and underdiagnosed. Magnetic resonance imaging findings such as spinal cord edema and dilated and tortuous perimedullary veins play a pivotal role in the confirmation of the diagnosis. However, spinal angiography remains the gold standard in the diagnosis of SDAVF. Classic angiographic findings of SDAVF are early filling of radicular veins, delayed venous return, and an extensive network of dilated perimedullary venous plexus. A series of angiograms of SDAVF at different locations along the spinal column, and mimics of serpentine perimedullary venous plexus on MR images, are demonstrated. Thorough knowledge of SDAVF aids correct diagnosis and prevents irreversible complications.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Central Nervous System Vascular Malformations/*diagnosis/epidemiology/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Spinal Cord Diseases/diagnosis ; Spine/radiography

Osler-Weber-Rendu disease is a rare autosomal dominant disorder of fibrovascular tissues, characterized by a classic triad of mucocutaneous telangiectasias, recurrent hemorrhages, and a familial occurrence. Portosystemic encephalopathy in a patient with Osler-Weber-Rendu disease is rare, but we experienced a case presenting with recurrent portosystemic encephalopathy in Osler-Weber-Rendu disease. We report on a case of a 75-year-old female presenting with an altered mentality. Initial studies including brain imaging study did not reveal any specific cause for her mental status. She was diagnosed with the rare disease after a series of tests and received conservative treatment. Her neurological status recovered fully without complication after conservative treatment and she was discharged after 18 hospital days. This case demonstrated an extremely rare case of Osler-Weber-Rendu disease presenting as portosystemic encephalopathy treated successfully with conservative treatment. For patients who have shown hepatic encephalopathy without a definite cause, we recommend evaluation for the possibility of Osler-Weber-Rendu disease. Conservative treatment based on treatment of advanced liver cirrhosis could be an alternative solution.
Aged ; Brain/diagnostic imaging ; Electroencephalography ; Female ; Hepatic Encephalopathy/*diagnosis ; Humans ; Magnetic Resonance Imaging ; Pedigree ; Telangiectasia, Hereditary Hemorrhagic/*diagnosis ; Tomography, X-Ray Computed ; Vascular Malformations/etiology

BACKGROUNDAlthough there were several clinical and experimental studies discussing the pathogenesis of dural arteriovenous fistula (DAVF), the pathological process leading to intracranial DAVF so far remains unknown. In this study, we investigated the expression of vascular growth factors in order to elucidate the possible role of these factors for the development of DAVF and to study the biological activity of this uncommon lesion.

METHODSWe examined the histological features, proliferative and angiogenic capacities of the tissue specimens obtained from 6 patients who underwent surgery at our institution. Immunohistochemical staining for vascular endothelial growth factor (VEGF), its receptors Flk-1 and Flt-1, ephrin-B2, MIB-1 and proliferating cell nuclear antigen (PCNA) was performed using standard immunohistochemical techniques.

RESULTSA positive immunostaining was found for all antibodies studied except MIB-1, whereas nuclear endothelial expression of PCNA was observed in only 3/6 cases. VEGF stained positive in all of the available specimens (6/6). Flk-1 showed a positive immunoreaction in only 2/6 cases and Flt-1 in 4/6 cases. Ephrin-B2 was expressed in the majority (5/6) of the cases.

CONCLUSIONSThese results support the hypothesis that DAVFs might be acquired dynamic vascular malformations with low biological activity. Vascular growth factors like VEGF and ephrin-B2 might play a pivotal role in the formation of DAVF.

Aged ; Cell Division ; Central Nervous System Vascular Malformations ; etiology ; metabolism ; pathology ; Endothelial Cells ; cytology ; Ephrin-B2 ; analysis ; physiology ; Female ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; analysis ; Male ; Middle Aged ; Neovascularization, Physiologic ; Proliferating Cell Nuclear Antigen ; analysis ; Vascular Endothelial Growth Factor A ; analysis

Double aortic arch with an atretic left arch distal to the origin of left subclavian artery was diagnosed with multi-detector row computed tomography (MDCT) in two children with dysphagia. This rare type of complete vascular ring is clinically important because it may be confused with right aortic arch in mirror imaging. Anatomic details of this rare type of complete vascular ring demonstrated on MDCT facilitated appropriate surgical treatment.
Adolescent ; Aorta, Thoracic/*abnormalities/radiography/surgery ; Child, Preschool ; Deglutition Disorders/etiology/radiography/surgery ; Female ; Humans ; Multidetector Computed Tomography/*methods ; Subclavian Artery/*abnormalities/radiography/surgery ; Vascular Malformations/complications/*radiography/surgery ; Vascular Surgical Procedures

OBJECTIVETo investigate the safe and effective treatment for painful venous malformation (VM) in limbs.

METHOD(1) 97 cases with painful VM underwent MRI to detect the location of VM, as well as its size and structure, its relationship with the surrounding tissue. Statistical analysis was also performed. (2) The embolic agent (ethanol) was first injected to embolize the draining vessels of VM, then the Polidocanol plus Methotrexate (MTX) was followed to keep the embolization effect on VM. The therapeutic effect was observed and analyzed.

RESULTSFrom January 2010 to January 2012, 97 patients with painful VM were treated. A Spearman correlation analysis showed no significant correlation between symptoms of pain and lesion growth, volume, or MRI grades (P > 0.05). The lesions in the muscle space are more likely to have the symptoms of pain (P < 0.01), followed by the lesions in the muscle, then the lesions in the joint and subcutaneous tissue. The pain relieve percentage was 95.9% (93/97) after one time embolic sclerotherapy. No severe complication, such as distant embolization, nerve damage, or muscle atrophy happened. No pain reoccurrence happened after 0.5-1.5 years of follow-up period.

CONCLUSIONSThe treatment of embolic scleratherapy is minimal invasive, safe and effective for painful VM with stable results.

Ethanol ; therapeutic use ; Extremities ; blood supply ; Humans ; Methotrexate ; therapeutic use ; Pain ; etiology ; Pain Management ; methods ; Polyethylene Glycols ; therapeutic use ; Sclerosing Solutions ; therapeutic use ; Sclerotherapy ; methods ; Statistics, Nonparametric ; Vascular Malformations ; complications ; pathology ; therapy ; Veins ; abnormalities

INTRODUCTIONWhile paediatric strokes are fairly uncommon, they are often associated with significant long-term disability. Diagnosis is often delayed because of the need to exclude conditions that mimic stroke. Understanding the outcomes related to stroke in children is important in the development of secondary prevention strategies. The aim of this study was to evaluate the epidemiology of childhood stroke in a tertiary paediatric unit in Singapore and to assess factors influencing outcome in these children.

MATERIALS AND METHODSA retrospective case-note review of all childhood strokes presenting to the Children's Medical Institute (CMI) at the National University Hospital (NUH), Singapore between October 1999 and May 2006. Data collected include demographic factors, clinical presentation, diagnosis, subsequent management and follow-up using specific outcome measures.

RESULTSTwenty-six children with a median age of 8.0 years at presentation were identified, comprising 15 ischaemic strokes (57.7%), 10 haemorrhagic strokes (38.5%) and 1 patient with both ischaemic and haemorrhagic lesions. The most common symptoms at presentation were seizures (15/26, 57.7%), lethargy (11/26, 42.3%), hemiparesis (10/26, 38.5%) and altered levels of consciousness (10/26, 38.5%). Vascular abnormalities accounted for 50% of strokes in our study population. The average length of follow-up was 33.2 months (range, 1 to 120) with only 11 children (11/26, 42.3%) achieving full recovery. Significant prognostic factors include altered consciousness and seizures at presentation, lesions in both cortical and subcortical locations, systemic disease aetiology, neurological deficits at discharge and seizures at the time of discharge.

CONCLUSIONLong-term neurological, neuropsychological and functional impairment are common in survivors of paediatric strokes. Certain clinical features and lesion characteristics are useful indicators of prognosis in these children.

Adolescent ; Age Factors ; Asian Continental Ancestry Group ; Brain Ischemia ; epidemiology ; etiology ; rehabilitation ; Cerebral Hemorrhage ; etiology ; Child ; Child, Preschool ; Developmental Disabilities ; etiology ; Female ; Humans ; Infant ; Intracranial Arteriovenous Malformations ; complications ; Length of Stay ; statistics & numerical data ; Male ; Neuropsychological Tests ; Prognosis ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Singapore ; epidemiology ; Stroke ; epidemiology ; etiology ; Stroke Rehabilitation ; Treatment Outcome ; Vascular Diseases ; complications

OBJECTIVE: We wanted to evaluate the safety and feasibility of ethanol sclerotherapy for treating craniofacial venous malformations (CVMs). MATERIALS AND METHODS: From May 1998 to April 2007, 87 patients (40 men and 47 women; age range, 2-68 years) with CVMs underwent staged ethanol sclerotherapy (range, 1-21 sessions; median number of sessions, 2) by the direct puncture technique. Clinical follow up (range, 0-120 months; mean follow up, 35 months; median follow up, 28 months) was performed for all the patients. Therapeutic outcomes were established by evaluating the clinical outcome of the signs and symptoms in all patients, as well as the degree of devascularization, which was determined on the follow-up imaging, in 71 patients. RESULTS: A total of 305 procedures with the use of ethanol were performed in 87 patients. Follow-up imaging studies were performed for 71 of 87 patients. Twenty-three (32%) of the 71 patients showed excellent outcomes, 37 patients (52%) showed good outcomes and 11 patients (16%) showed poor outcomes. Ethanol sclerotherapy was considered effective for 60 patients. All the minor complications such as bulla (n = 5) healed with only wound dressing and observation. Any major complication such as skin necrosis did not develop. CONCLUSION: Percutaneous ethanol sclerotherapy is an effective, safe treatment for CVMs.
Adolescent ; Adult ; Aged ; Analgesics/administration & dosage ; Anti-Infective Agents, Local/adverse effects/*therapeutic use ; Child ; Child, Preschool ; Craniofacial Abnormalities/*therapy ; Ethanol/adverse effects/*therapeutic use ; Feasibility Studies ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Pain/drug therapy/etiology ; Retrospective Studies ; Sclerosing Solutions/adverse effects/therapeutic use ; Sclerotherapy/adverse effects/*methods ; Treatment Outcome ; Vascular Malformations/*therapy ; Young Adult