scholarly journals Orbital Lymphatic-Venous Malformation Accompanied by an Intraocular Vascular Malformation: A Rare Case Study

2021 ◽  
pp. 396-401
Author(s):  
Karny Shouchane-Blum ◽  
Iftach Yassur ◽  
Amir Sternfeld ◽  
Miriam Regev ◽  
Orly Gal-Or ◽  
...  

Lymphatic-venous malformations (LVMs) are development defects that result in abnormal connections between the lymphatic and venous systems. The authors describe a 7-weeks-old female infant who presented with a right orbital LVM extending to the ipsilateral cheek and subconjunctiva of the right eye, intracranial developmental venous anomalies in the right cerebellum, and a significant right eye intraocular retinal vascular malformation. Since orbital LVM is usually diagnosed in infancy or childhood, pediatric ophthalmologists should actively look for intraocular vascular malformations as such findings can poorly affect a patient’s vision.

2003 ◽  
Vol 9 (2) ◽  
pp. 169-176 ◽  
Author(s):  
J.I. Chung ◽  
H. Alvarez ◽  
P. Lasjaunias

We report a sporadic case of probable BRBN (blue rubber bleb nevus syndrome) with multiple CNS (central nervous system) involvement. These features consisted of multiple VMs (venous malformations) and DVAs (developmental venous anomalies) in supratentorial brain, cerebellum, and diencephalon. Since its first description by Bean, there have been many cases of BRBN manifesting with gastrointestinal bleeding with or without associated hemorrhage. Cases with CNS involvement were rarely reported and many of the descriptions were confusing with different terminologies used to describe them such as capillary venous malformation, hemangiomas, and vascular malformations. The lesions illustrated are venous malformations similar to our case. The association of DVA was recognized in some cases; they are likely to be underestimated when revisiting the published case illustrations. Although our case is sporadic, the link with HHT1 is unlikely despite the involvement of the same chromosome (Ch 9).


2021 ◽  
pp. 1-4
Author(s):  
Serhat Yarar ◽  
Ilker Uyar ◽  
Mehmet Emin Cem Yildirim ◽  
Mehmet Dadacı ◽  
Bilsev Ince

Primary intraosseous vascular malformations (PIVMs) are rare intraosseous lesions, accounting for approximately 0.5–1% of all intraosseous tumours. In this case report, we aimed to present a rare case of intraosseous vascular malformation causing a large lytic area in the parietal bone. A 25-year-old male patient was admitted to the clinic with a mass on the parietal bone. On physical examination, it was observed that the hair density on the mass was decreased, the mass had a soft consistency, and there was no pain on palpation. The patient was operated under local anaesthesia with a provisional diagnosis of a trichilemmal cyst. However, intraoperative diagnosis was a vascular malformation. There was a 3-cm full-thickness defect on the parietal bone caused by the lesion. The mass was excised completely while preserving the integrity of the dura. The resulting defect was reconstructed with bilateral rotation advancement flaps. The calvarial defect was not reconstructed due to equipment inadequacy. No complications were encountered in the postoperative period. Ninety-three PIVM cases have been reported in the skull since 1845. In very few of these cases, the mass is located in the parietal bone. The pathogenesis of PIVMs is not completely understood. The definitive diagnosis is made by histopathological examination. The therapeutic gold standard is surgery. Surgeons should keep in mind that radiological examination before the operation could prevent undesirable complications.


2016 ◽  
Vol 29 (6) ◽  
pp. 465-469 ◽  
Author(s):  
Nitin Agarwal ◽  
Giulio Zuccoli ◽  
Geoffrey Murdoch ◽  
Brian T Jankowitz ◽  
Stephanie Greene

Introduction Developmental venous anomalies (DVAs) are cited as the most common cerebral vascular malformations. Still, intracerebral hematomas are rarely thought to be caused by DVAs. In this report, the authors present a unique case of a DVA that hemorrhaged spontaneously, rather than hemorrhaging into a venous infarction following DVA thrombosis as has been more commonly reported. Clinical presentation A 22-year-old previously healthy male presented to the emergency department with a severe headache, confusion, and progressive hemiparesis. A computed tomography (CT) scan demonstrated a spontaneous left parietal intraparenchymal hemorrhage (IPH), with intraventricular extension and acute hydrocephalus. CT angiography did not demonstrate an underlying vascular malformation. The patient was taken emergently to the operating room for a left parietal craniotomy for evacuation of the hematoma. Intraoperative pathology was consistent with a DVA Postoperative magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and magnetic resonance venography (MRV) did not demonstrate a mass lesion, ischemic stroke, or underlying vascular malformation. An MRI obtained three years previously for headaches was normal. A postoperative diagnostic cerebral angiogram was normal. An MRI/MRA performed six months postoperatively demonstrated two foci of abnormal vessels on susceptibility-weighted imaging (SWI), suggesting the presence of a venous vascular malformation. A diagnostic cerebral angiogram obtained six months postoperatively was again normal, including delayed imaging. Conclusion Few reports have cited DVA as the sole cause of intracerebral hemorrhage. While very rare, these reports suggest hemorrhagic conversion of a venous infarction secondary to a thrombosed DVA as a possible etiology, and several provide imaging consistent with this diagnosis. This case study demonstrates a unique presentation of a hemorrhagic DVA in the absence of thrombosis or stroke.


2014 ◽  
Vol 2014 ◽  
pp. 1-2 ◽  
Author(s):  
Dilek Sarici ◽  
Mustafa Ali Akin ◽  
Selim Kurtoglu ◽  
Filiz Tubas ◽  
Serdar Umit Sarici

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet).


2018 ◽  
Vol 36 (1) ◽  
pp. 83
Author(s):  
Nawaporn Assanangkornchai ◽  
Kumpol Aiempanakit ◽  
Siripan Sangmala

Klippel-Trenaunay syndrome (KTS) is a rare, vascular malformation. The major clinical presentation is an overgrowth involving the extremities. The diagnosis of KTS was based on an imaging study, which revealed vascular malformations. The author’s report is of a 36-year Thai man with; hypertrophy of the right lower extremity, whilst having suffered from two-months of bloody defecation. The magnetic resonance imaging showed venous malformations with soft tissue hypertrophy of the affected limb, genitalia and rectum. The patient was diagnosed with: KTS accompanied by gastrointestinal complications.


2021 ◽  
pp. 80-82
Author(s):  
B.V Subhash ◽  
Alba Dinesh ◽  
Seema Patil ◽  
Asha R Iyengar ◽  
Revan Kumar Joshi ◽  
...  

The vascular anomalies consist of vascular tumours and vascular malformations. One among these are the venous malformations which constitute about 40% of all vascular malformations. Usually, the venous malformations present as localized lesions in the form of swellings/nodules with phleboliths, are diagnosed by ultrasonography, CT and MRI. Summary: This is a report of a rare longstanding case of wide spread venous malformations of oral cavity and facial area with phleboliths.


2016 ◽  
Vol 125 (1) ◽  
pp. 167-172
Author(s):  
Sergei Terterov ◽  
Nancy McLaughlin ◽  
Harry Vinters ◽  
Neil A. Martin

Angiographically occult cerebral vascular malformations (AOVMs) are usually found in the supratentorial brain parenchyma. Uncommonly, AOVMs can be found within the cavernous sinus or basal cisterns and can be associated with cranial nerves. AOVMs involving the intracranial segment of the spinal accessory nerve have not been described. A 46-year-old female patient presented with a history of episodic frontal headaches and episodes of nausea and dizziness, as well as gait instability progressing over 6 months prior to evaluation. Imaging revealed a well-circumscribed 3-cm extraaxial T1-weighted isointense and T2-weighted hyperintense contrast-enhancing mass centered in the region of the right lateral cerebellomedullary cistern. The patient underwent resection of the lesion. Although the intraoperative appearance was suggestive of a cavernous malformation, some histological findings were atypical, leading to the final diagnosis of vascular malformation, not otherwise specified. The patient’s postoperative course was uneventful with complete resolution of symptoms. To the authors’ knowledge, this is the first report of an AOVM involving the intracranial portion of the accessory nerve. For any AOVM located within the cerebellomedullary cistern or one suspected of involving a cranial nerve, the authors recommend including immunohistochemistry with primary antibody to neurofilament in the histopathology workup.


2019 ◽  
Vol 29 (5) ◽  
pp. 717-719
Author(s):  
Jun Sato ◽  
Atsuko Kato ◽  
Naoki Ohashi

AbstractIsolation of the left innominate artery from the right aortic arch is a rare anomaly. Herein, we present an even rarer case of incomplete isolation of the proximal left innominate artery with the right aortic arch in a 3-month-old female infant with a double-outlet right ventricle and pulmonary stenosis. Surgical repair at 6 months of age was successful, leading to the restoration of adequate flow in the left arm.


2020 ◽  
Vol 03 (01) ◽  
pp. e10-e15
Author(s):  
Yuki Sato ◽  
Takao Hamamoto ◽  
Takashi Ishino ◽  
Tsutomu Ueda ◽  
Masay Takumida ◽  
...  

AbstractVenous malformations commonly occur in the head and neck regions, in the mucous membrane of the nasal and oral cavities. Venous malformations are found in the extremities in 40% of cases, in the trunk in 20% of cases, and in the cervicofacial area in 40% of cases. However, they are rarely encountered in the parapharyngeal space. We report our experience of surgical treatment of a patient with this rare tumor. The patient was a 21-year-old man who presented with a left mandibular swelling and was admitted to a nearby hospital. He was referred to our hospital for examination and treatment. Contrast-enhanced computed tomography (CT) revealed a bulky tumor with some calcification in the parapharyngeal space; on contrast-enhanced CT, the tumor showed some enhanced effects in the arterial and venous phases. Additional enhancement magnetic resonance imaging (MRI) showed the lesion as low signal intensity on T1-weighted images and as high signal intensity on T2-weighted images, and contrast-enhanced MRI showed some enhanced effects of the lesion. Based on these findings, we suspected venous malformation and performed surgical excision. Histopathology confirmed the venous malformation, and the lesion was diagnosed as a cavernous angioma. Vascular malformations in the head and neck regions should be precisely diagnosed so that appropriate multimodality treatment can be undertaken.


2007 ◽  
Vol 22 (6) ◽  
pp. 249-252 ◽  
Author(s):  
B B Lee ◽  
J Laredo ◽  
T S Lee ◽  
S Huh ◽  
R Neville

Venous malformation (VM) is a congenital vascular malformation (CVM) that develops along the venous system through the various stages of embryogenesis. Older terminology and classification were often misleading and confusing. A newer classification system has emerged that is based on advances in the study of these conditions and is useful in contemporary management. The Hamburg classification was introduced after reappraisal of older terminology and has become the standard system for contemporary classification, which is based on anatomical, pathological and embryological criteria.


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