Successful embolization of a primary bronchial artery racemose hemangioma by the ingenious use of a microballoon catheter.

Primary racemose hemangioma of the bronchial artery (RHBA) is a rare congenital condition characterized by enlarged and convoluted bronchial arteries, often forming fistulas between the pulmonary artery or vein. Herein, we report a case of balloon-assisted embolization using N-butyl-2-cyanoacrylate (NBCA) performed for a primary RHBA in an asymptomatic 57-year-old woman. There were 2 tortuous and dilated branches of the right bronchial artery (RBA) (arising from the left subclavian artery and the descending thoracic aorta) which merged in the right pulmonary hilar region, forming multiple aneurysms that short-circuited the right pulmonary artery. Embolization was performed via the RBA arising from the subclavian artery using a triaxial system (a 5-Fr guiding catheter, a 2.7-Fr microballoon catheter, and a 1.9-Fr microcatheter). The guiding catheter was inserted into the peripheral side of the RBA using the microballoon catheter as an anchor; the balloon was slightly inflated and advanced further into the peripheral side using blood flow. The microcatheter was inserted as close to the aneurysms as possible and embolization was done using an NBCA-iodized oil mixture under blood flow control by the microballoon catheter. Bronchial artery embolization is the treatment of choice for primary RHBA because of the risk of rupture, and balloon-assisted techniques can help guide the catheter distally while also controlling blood flow.

Racemose hemangioma presenting as a vitreous hemorrhage: case report / Hemangioma racemoso apresentando-se como hemorragia vítrea: relato de caso

ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.

RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.

Iris Racemose Hemangioma Assessment with Swept Source Optical Coherence Tomography Angiography: A Feasibility Study and Stand-Alone Comparison.

PURPOSE: To evaluate arteriovenous malformations (AVM) with swept-source (SS) optical coherence tomography (OCT) angiography (OCTA) in iris racemose hemangioma and compare it with traditional intravenous iris fluorescein angiography (IVFA). METHODS: A cross-sectional observational clinical study was conducted on patients with iris racemose hemangioma with the ZEISS PLEX Elite 9000 SS OCT & OCTA. RESULTS: Three eyes of three patients were imaged. Iris racemose hemangiomas demonstrated a tortuous, well-defined, and continuous course of the AVM. The ZEISS PLEX Elite 9000 SS OCT & OCTA allowed for a detailed visualization of the ARM and was superior to IVFA in depicting small caliber, fine vessels. CONCLUSIONS: SS-OCTA may provide a dye-free, no-injection, cost-effective method comparable to spectral domain OCTA and IVFA for diagnosing and monitoring iris racemose hemangiomas for growth and vascularity.

A case of primary racemose hemangioma with endobronchial lesions demonstrating recurrent hemoptysis initially treated with bronchial arterial embolization.

Primary racemose hemangioma of the bronchial artery (RHBA) is one of the causes of massive hemoptysis. A 72-year-old woman was admitted to our hospital with recurrent hemoptysis. Bronchoscopy showed an endobronchial lesion, and the angiography of the right bronchial arteries indicated RHBA. Bronchial arterial embolization (BAE) was performed to prevent hemoptysis. Although the endobronchial lesion shrank after the first BAE, the lesion re-increased and caused massive hemoptysis. A thoracoscopic right upper lobectomy was performed, and hemoptysis did not recur. Therefore, in cases of RHBA where there is recurrent hemoptysis and the endobronchial lesions that remain after BAE, additional treatments should be considered.

Huge racemose hemangioma of the bronchial artery with arterial supply via the right coronary artery and left internal thoracic artery.

A 79-year-old male with bronchiectasis was referred to our clinic because of mild chest tightness on exertion. He had no history of hemoptysis. An electrocardiogram showed ST segment depression in leads V5-6. Multi-detector contrast-enhanced computed tomography revealed no significant stenosis in either coronary artery; however, a huge racemose hemangioma of the bronchial artery (RHBA) was detected. In addition, arterial supply to the RHBA via the right coronary artery (RCA) and the left internal thoracic artery (LITA) was suspected. Adenosine-loading myocardial scintigraphy images revealed segmental hypo-perfusion in the left ventricular inferior wall. Selective bronchial artery angiography revealed the huge RHBA. In addition, both the RCA and LITA provided arterial supply to the RHBA. To the best of our knowledge, this case is the first to show multiple arterial supply resulting in a huge RHBA.

A Surgical Case of Bronchial Artery Aneurysm Connecting to a Pulmonary Artery and Vein Complicated by Racemose Hemangioma.

We report a surgical case of bronchial artery aneurysm (BAA) that directly connected to a pulmonary artery and a pulmonary vein through an abnormal vessel. It was complicated by racemose hemangioma. This is a rare vascular malformation. An 82-year-old female had a large BAA that was found incidentally. First, we consider treating the BAA with embolization by interventional radiology (IVR). However, because of strong meandering of the bronchial artery, we could not advance a microcatheter into the BAA. Therefore, a surgical operation was performed through a standard posterior lateral thoracotomy. The BAA was located between the upper and lower lobes and directly connected to the pulmonary artery. Some bronchial artery branches that provided inflow to the aneurysm were ligated, and the abnormal vessel that connected the BAA to the upper pulmonary vein was ligated easily. A fistula between the BAA and pulmonary artery was sutured by the cardiovascular surgeon using an artificial cardiopulmonary device, with permissive stenosis of A2b (ascending A2).

Retinal Racemose Hemangioma With Optic Neuropathy in a Child.

The aim of this study is to report an unusual case of retinal racemose haemangioma (RRH) in a child resulting in optic neuropathy and its optical coherence tomography angiography (OCT-A) findings. This is a retrospective case report. For almost a year, a 13-year-old girl experienced gradual, painless, generalized blurred vision in her right eye. Visual acuity was 6/60 with a positive relative afferent pupillary defect (RAPD) in her right eye. The right-eye fundus showed enlarged and tortuous retinal vessels extending from the optic disc to all four quadrants, including the juxta foveal region. OCT analysis revealed distortion in the region of enlarged vessels with minimal retinal fluid while OCT-A of the macula area demonstrated dilated and tortuous vessels in the superficial layers of the retina. Right intra-orbital vascular channels surrounding the optic nerve with optic nerve atrophy and gliosis were detected on magnetic resonance imaging angiography/venography (MRA/MRV). The cerebral angiogram reported an abnormal tangle of small vessels within the right orbit that received supply from a dilated right ophthalmic artery indicating the presence of retro-orbital arteriovenous malformation (AVM). She was then referred to the neurosurgeon and a decision was made not to embolize or resect the dilated vessel as this might lead to occlusion of the ophthalmic artery and thus worsen her vision. RRH may present in the paediatric age group, and optic nerve atrophy is one of the disease manifestations. OCT-A is a less invasive diagnostic option compared to fundus fluorescein angiography (FFA) for diagnosis and monitoring of disease progression.

A case of primary racemose hemangioma in which the disappearance of an endobronchial lesion was confirmed after bronchial artery embolization.

The confirmation of the improvement of endobronchial lesions in addition to that of vascular lesions after bronchial artery embolization of primary racemose hemangioma could be important.

Racemose hemangioma complicated with macular macroaneurysm rupture.

PURPOSE: To describe a rare case of racemose hemangioma which developed spontaneous macular macroaneurysm (MA) rupture and vitreaous hemorrhage. OBSERVATIONS: A 29-year-old healthy asian female visited our hospital and a racemose hemangioma was found in the left eye. At presentation, the best corrected visual acuity (BCVA) was 30/20 in her left eye. At 9 years after the first visit, MA-like lesion was noted in the macular area. After that, vitreous and subretinal hemorrhage appeared in the left eye. The patient underwent simultaneous vitrectomy and cataract surgery, but vitreous re-hemorrhage occurred two days after the operation. To avoid re-hemorrhage, silicone oil (SO) tamponade was added in the second vitrectomy. Two years after the second operation, SO was removed and postoperative BCVA in the left eye was 20/200 without re-bleeding in the vitreous. CONCLUSIONS AND IMPORTANCE: Although retinal hemorrhages have been reported in the patients with a racemose hemangioma, in our case the macular MA rupture occurred at 9 years after the first visit. Congenital retinal arteriovenous anastomosis can show a change in vascular shape in some cases, thus it is important to observe carefully.

Periocular steroids for macular edema associated with retinal arteriovenous malformation: A case report.

Retinal racemose hemangiomas (RRH) are vascular malformations comprising of direct arteriovenous communications in the retina. Exudation and neurosensory detachment are some of the complications which may cause decreased visual acuity. Herein, we describe a case of a 38-year-old male presenting with unilateral Group II RRH complicated with macular edema. Initial treatment with intravitreal bevacizumab yielded a poor therapeutic response. Subsequently, he was treated with a posterior sub-tenon injection of triamcinolone acetonide following which there was a prompt decrease in edema with simultaneous improvement in vision. The visual acuity was maintained and no recurrence was seen even after 6 months of successful treatment.

Racemose hemangioma of the bronchial artery mimicking esophageal submucosal tumor: a case report.

Primary racemose hemangioma of the bronchial artery is a rare congenital disease that is occasionally complicated by aneurysms. An asymptomatic 78-year-old man was referred to our hospital with an esophageal submucosal tumor that was endoscopically found in the upper thoracic esophagus in a health check-up. Physical examination findings were unremarkable. Contrast-enhanced chest computed tomography with three-dimensional image reconstruction and selective bronchial arteriography resulted in a definitive diagnosis of primary racemose hemangiomas of the bronchial arteries accompanied by left bronchial artery aneurysm. Because rupture of a bronchial artery aneurysm can cause critical life-threatening hemorrhage, bronchial arterial embolization using coils and a mixture of N-butyl-2-cyanoacrylate and iodized oil was thus performed for bronchial artery aneurysm. Postoperative course was uneventful, and the patient was discharged on the third postoperative day. Computed tomography performed after 6 months revealed no enhancement of the aneurysms. In conclusion, we report a case of an asymptomatic primary racemose hemangioma of the bronchial artery accompanied by an aneurysm that mimicked a submucosal esophageal tumor. We also reviewed other Japanese case of primary racemose hemangioma of the bronchial artery accompanied by aneurysm based on the literature.

Optical coherence tomography of racemose angioma.

Wyburn-Mason syndrome is associated with racemose angioma of the retina and arteriovenous malformation of the brain. Optical coherence tomography and MRI angiography may be used to document the vascular lesion of the retina and brain, respectively.

Successful transcatheter arterial embolization in an asymptomatic patient with primary racemose hemangioma of the bronchial artery.

An asymptomatic 70-year-old woman presented with a nodular lesion overlapping the pulmonary artery at the right hilar region on a chest X-ray. Bronchial arteriography revealed an aneurysmal dilation of the long segment of the right bronchial artery and a shunt from the right bronchial artery to the right lower pulmonary artery. She was diagnosed with primary racemose hemangioma of the bronchial artery (RHBA). Considering the risk of hemoptysis, we performed a bronchial arterial embolization (BAE) using coils and N-butyl-2-cyanoacrylate. She had no complication after the BAE and no recurrences of hemoptysis at the 36-month follow-up. RHBA should be considered in case of aneurysmal dilation in the long segment of the bronchial artery, and BAE should be considered as a treatment strategy despite the absence of symptoms.

Successful Treatment of Bronchial Artery Aneurysms Associated With Racemose Hemangioma by Combined Therapy of Transcatheter Bronchial Artery Embolization and Thoracic Endovascular Aortic Repair.

A 70-year-old man was referred to our hospital for an abnormal chest shadow. Enhanced computed tomography (CT) revealed 2-humped bronchial artery aneurysms (BAAs) associated with racemose hemangioma. The combined therapy of transcatheter bronchial artery embolization and thoracic endovascular aortic repair was performed. Postoperative CT confirmed the complete exclusion of the aneurysms with no evidence of an endoleak. Our result suggests that this combined therapy is a safe and effective treatment for BAA.

Small bowel racemose hemangioma complicated with obstruction and chronic anemia: A case report and review of literature.

BACKGROUND: Gastrointestinal hemangiomas are rare benign tumors. According to the size of the affected vessels, hemangiomas are histologically classified into cavernous, capillary, or mixed-type tumors, with the cavernous type being the most common and racemose hemangiomas being very rare in the clinic. Melena of uncertain origin and anemia are the main clinical manifestations, and other presentations are rare. Due to the rarity of gastrointestinal hemangiomas and lack of specific manifestations and diagnostic methods, preoperative diagnoses are often delayed or incorrect. CASE SUMMARY: We report a 5-year-old girl who presented with abdominal pain, nausea, and vomiting for a duration of 10 h. The laboratory studies showed prominent anemia. Computed tomography and contrast-enhanced computed tomography of the abdomen revealed a small bowel obstruction caused by a giant abdominal mass. Segmental resection of the ileal lesions was performed through surgery, and the final pathology results revealed a diagnosis of racemose hemangioma complicated by a small bowel obstruction and simultaneous chronic anemia. CONCLUSION: The current report will increase the understanding of the diagnosis and treatment of gastrointestinal hemangiomas and provide a review of the related literature.

Wyburn-Mason syndrome presenting with bilateral retinal racemose hemangioma with unilateral serous retinal detachment.

Wyburn-Mason syndrome is associated with unilateral retinal racemose hemangioma. Rarely, it presents with bilateral and symmetrical grade of malformation. We describe a 37-year old male, who presented with Wyburn-Mason syndrome presenting with bilateral but asymmetrical retinal hemangioma. The eye with advanced grade of hemangioma was complicated with exudation, intraretinal fluid, neurosensory detachment, and reduced vision. He was treated with one intravitreal injection of bevacizumab, after which both the intraretinal fluid and neurosensory detachment resolved. His vision improved and was maintained till 1 year of follow-up.

Retinal racemose hemangioma with retinal artery macroaneurysm: Optical coherence tomography angiography (OCTA) findings.

PURPOSE: To describe a rare case of retinal racemose hemangioma (RRH) with retinal artery macroaneurysm (RAM) and its optical coherence tomography angiography (OCT-A) finding before and after treatment. OBSERVATIONS: Congenital arteriovenous (AV) communications or RRH is a rare developmental anomaly associated with various ocular conditions. RRH alone is usually asymptomatic, and vision loss occurs when associated with other ocular complications like retinal vein occlusion, hemmorage, macular edema and macroaneurysm. In this communication we describe a case of a 44-year-old female having RRH who presented with active, leaking RAM with subretinal hemmorage. OCT-A through the lesion demonstrated an active aneurysm which on subsequent treatment with anti VEGF and focal laser photocoagulation showed involution of aneurysm. CONCLUSIONS AND IMPORTANCE: RRH is usually asymptomatic and may be associated with vision threatening ocular complications like RAM. OCT-A helps in giving additional dynamic blood flow information in RAM.

Primary racemose hemangioma with bronchial-pulmonary arterial fistula.

Primary racemose hemangioma with bronchial-pulmonary arterial fistula is a very rare abnormality. We herein report an asymptomatic case of primary racemose hemangioma with no significant size change in 5 years.

Multiple Enlarged Aneurysms in Primary Racemose Hemangioma of the Bronchial Artery: Successful Prophylactic Transcatheter Arterial Embolization Using N-butyl-2-cyanoacrylate and Coils.

An asymptomatic 48-year-old man presented with multiple aneurysms in a primary racemose hemangioma of the right bronchial artery. Bronchial arteriography revealed a tortuous artery with four fusiform aneurysms of varying sizes and aneurysmal dilatation with marked thrombus formation in the long segment of the distal portion. Because the tip of catheter could not pass beyond the aneurysmal dilatation, we performed balloon-occluded embolization using a mixture of N-butyl-2-cyanoacrylate (NBCA) and iodized oil. For four other aneurysms, we performed embolization using a coil alone or with NBCA. After 6 months, right bronchial arteriography revealed no enhancement of the aneurysms. Despite the rarity of this procedure, embolization with NBCA is a good option for bronchial artery aneurysm embolization.