• Tuberculosis and Respiratory Diseases
• /
• v.45 no.4
• /
• pp.896-901
• /
• 1998

Pulmonary arteriovenous malformation(PAVM) is an uncommon congenital anomaly. As pulmonary arteriovenous malformation is a direct communication between the branches of pulmonary artery and vein which originated from the malformation of capillary development, major disturbances in gas exchange can result. This malformation results in the several symptoms such as dyspnea, hemopyssis, cyanosis, and severe neurologic complaints. However, the most of patients are usually asymptomatic. Selective pulmonary angiography is well known the helpful diagnostic method. Recently, therapeutic embolization has been advocated as the treatment of choice for pulmonary arteriovenous malformations. We report a case of multiple pulmonary arteriovenous malformation, which was detected on the simple chest X-ray and successfully treated with coil embolization in a 19-year-old asymptomatic woman.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.11 no.2
• /
• pp.198-203
• /
• 2008

We report a 10-year-old girl with the blue rubber bleb nevus syndrome (BRBNS) who had chronic severe anemia caused by chronic occult bleeding in the gastrointestinal (GI) tract. The patient was admitted to the hospital frequently for recurrent pallor and fatigue since the age of 7 years. Gastroduodenoscopy and capsule endoscopy revealed multiple venous malformations with blood oozing in the stomach, small bowel and colon. The patient was treated by aggressive surgical resection of the 23 vascular malformations in the GI tract. The patient is well without anemia 15 months post surgery.

• Imaging Science in Dentistry
• /
• v.44 no.1
• /
• pp.67-73
• /
• 2014

Intraosseous vascular lesions of the maxillofacial region are rare, and the differential diagnosis of intraosseous vascular malformations from other jaw lesions can be challenging. In the present case, magnetic resonance imaging and three-dimensional computed tomographic angiography (CTA) was used for diagnosis, and the lesion was treated wih surgical excision. Diverse characteristics such as the "honeycomb" and "sunburst" radiographic appearances and the absence of major peripheral feeder vessels in the CTA were noted. Intraosseous vascular malformations have a varied radiographic appearance, and the nomenclature of these lesions is equally diverse, with several overlapping terms. Pathologists do not generally differentiate among intraosseous vascular lesions on the basis of histopathology, although these lesions may present with contrasting immunohistochemical and clinical behaviors requiring varied treatment strategies. This case report highlights the need for multiple imaging modalities to differentiate among vascular lesions, as well as to better understand the behaviors of these unique lesions.

• Archives of Craniofacial Surgery
• /
• v.24 no.5
• /
• pp.236-239
• /
• 2023

Porocarcinoma (PC) and basal cell carcinoma (BCC) are distinct skin cancers. Few studies have documented the occurrence of two concurrent types of skin cancers, and to the best of our knowledge, this represents the inaugural report of such a coexisting lesion arising from a capillary malformation. Herein, we report a case of concurrent PC and BCC presenting with capillary malformation. A 93-year-old woman visited our hospital with a protruding mass in her right nasal ala that appeared as a capillary malformation. A biopsy was performed on the skin lesion, and BCC was diagnosed. A wide excision was performed. Permanent biopsy revealed that the skin lesion was a PC with basal cells and squamous differentiation. The safety margin of the deep tissue margin was < 0.1 cm; however, considering the advanced age of the patient, further excision was deemed to not possess any benefits. This case illustrates the importance of recognizing the possibility of multiple skin cancers, even in patients with benign lesions such as capillary malformations. The rarity of this presentation highlights the importance of thorough investigation and histopathological examination of skin lesions in guiding appropriate surgical excision.

• Tuberculosis and Respiratory Diseases
• /
• v.66 no.4
• /
• pp.314-318
• /
• 2009

Hereditary hemorrhagic telangiectasia (HHT, also called Osler-Weber-Rendu Disease) is a rare systemic fibrovascular dysplasia characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVMs). HHT is an autosomal dominant disease with a prevalence of 1 in 5,000~8,000. Recurrent epistaxis is often the first and most common manifestation, and about 30% of patients reveal pulmonary AVM. Presently, we report a familial case of HHT. A 61-year-old male with asymptomatic multiple pulmonary AVMs was successfully treated with embolization. His older brother who presented with recurrent epistaxis and multiple telangiectasias was treated with laser ablation. Their pedigree revealed a family history of recurrent epistaxis.

• BMB Reports
• /
• v.49 no.5
• /
• pp.255-262
• /
• 2016

The disease known as cerebral cavernous malformations mostly occurs in the central nervous system, and their typical histological presentations are multiple lumen formation and vascular leakage at the brain capillary level, resulting in disruption of the blood-brain barrier. These abnormalities result in severe neurological symptoms such as seizures, focal neurological deficits and hemorrhagic strokes. CCM research has identified 'loss of function' mutations of three ccm genes responsible for the disease and also complex regulation of multiple signaling pathways including the WNT/β-catenin pathway, TGF-β and Notch signaling by the ccm genes. Although CCM research is a relatively new and small scientific field, as CCM research has the potential to regulate systemic blood vessel permeability and angiogenesis including that of the blood-brain barrier, this field is growing rapidly. In this review, I will provide a brief overview of CCM pathogenesis and function of ccm genes based on recent progress in CCM research.

• Communications for Statistical Applications and Methods
• /
• v.29 no.2
• /
• pp.239-250
• /
• 2022

In many applications, we frequently encounter correlated multiple outcomes measured on the same subject. Joint modeling of such multiple outcomes can improve efficiency of inference compared to independent modeling. For instance, in developmental toxicity studies, fetal weight and number of malformed pups are measured on the pregnant dams exposed to different levels of a toxic substance, in which the association between such outcomes should be taken into account in the model. The number of malformations may possibly have many zeros, which should be analyzed via zero-inflated count models. Motivated by applications in developmental toxicity studies, we propose a Bayesian joint modeling framework for continuous and count outcomes with excess zeros. In our model, zero-inflated Poisson (ZIP) regression model would be used to describe count data, and a subject-specific random effects would account for the correlation across the two outcomes. We implement a Bayesian approach using MCMC procedure with data augmentation method and adaptive rejection sampling. We apply our proposed model to dose-response analysis in a developmental toxicity study to estimate the benchmark dose in a risk assessment.

• Radiation Oncology Journal
• /
• v.9 no.2
• /
• pp.351-359
• /
• 1991

The current LINAC technique for radiosurgery utilizes a single isocenter approach with multiple noncoplanar arcs. This approach results in spherical dose distributions in the target. Many arteriovenous malformations and tumors suitable for radiosurgical treatment have non-spherical or irregular shapes. The basic approach presented in this paper is to use two or multiple isocenters with standard arcs to shape irregular target volumes through the use of multiple spherical targets. Selection of reasonable irradiation parameters in the first stage is critical to the success of real-time optimization. A useful guideline for optimum isocenter separation and collimator size is developed to shape the target margin uniformly with an desired isodose surface for an elongated target. The implementation of multiple isocenters with three dimensional dose model and application of multiple isocenters approach to several cases are discussed.

• Korean Journal of Radiology
• /
• v.23 no.8
• /
• pp.794-802
• /
• 2022

Objective: To evaluate the feasibility of single-shot whole thoracic time-resolved MR angiography (TR-MRA) to identify the feeding arteries of pulmonary arteriovenous malformations (PAVMs) and reperfusion of the lesion after embolization in patients with multiple PAVMs. Materials and Methods: Nine patients (8 females and 1 male; age range, 23-65 years) with a total of 62 PAVMs who underwent percutaneous embolization for multiple PAVMs and were subsequently followed up using TR-MRA and CT obtained within 6 months from each other were retrospectively reviewed. All imaging analyses were performed by two independent readers blinded to clinical information. The visibility of the feeding arteries on maximum intensity projection (MIP) reconstruction and multiplanar reconstruction (MPR) TR-MRA images was evaluated by comparing them to CT as a reference. The accuracy of TR-MRA for diagnosing reperfusion of the PAVM after embolization was assessed in a subgroup with angiographic confirmation. The reliability between the readers in interpreting the TR-MRA results was analyzed using kappa (κ) statistics. Results: Feeding arteries were visible on the original MIP images of TR-MRA in 82.3% (51/62) and 85.5% (53/62) of readers 1 and 2, respectively. Using the MPR, the rates increased to 93.5% (58/62) and 95.2% (59/62), respectively (κ = 0.760 and 0.792, respectively). Factors for invisibility were the course of feeding arteries in the anteroposterior plane, proximity to large enhancing vessels, adjacency to the chest wall, pulsation of the heart, and small feeding arteries. Thirty-seven PAVMs in five patients had angiographic confirmation of reperfusion status after embolization (32 occlusions and 5 reperfusions). TR-MRA showed 100% (5/5) sensitivity and 100% (32/32, including three cases in which the feeding arteries were not visible on TR-MRA) specificity for both readers. Conclusion: Single-shot whole thoracic TR-MRA with MPR showed good visibility of the feeding arteries of PAVMs and high accuracy in diagnosing reperfusion after embolization. Single-shot whole thoracic TR-MRA may be a feasible method for the follow-up of patients with multiple PAVMs.

• Journal of the Korean Society of Radiology
• /
• v.82 no.6
• /
• pp.1628-1633
• /
• 2021

Spinal epidural arteriovenous fistulas (SEDAVFs) are rare spinal vascular malformations that are difficult to diagnose and treat. SEDAVFs can be asymptomatic; however, symptoms can arise from the compression of adjacent nerve roots by dilated vein and perimedullary venous reflux, caused by shunting into the epidural venous plexus. A 31-year-old male presented to our institution with a 2-year history of progressively worsening low-back pain, radiating thigh pain, and sensory changes in his lower extremities. MRI and CT angiography demonstrated dilated epidural vascular lesion compressing the nerve root. The SEDAVF was embolized with multiple coils, which alleviated the nerve root compression from the engorged venous varix and improved the patient's radiculopathy. Our experience from this case shows that endovascular coil embolization using the transarterial approach can be an effective treatment for SEDAVF and an alternative to surgical ligations.