Nasal Venous Malformation with Intracranial Communication: Presentation, Management, and Review of the Literature

Location

Medical Education Building, LSUHSC-NO

Presentation Date

10-10-2019 10:00 AM

End Date

10-10-2019 12:00 PM

Description

Introduction We report a rare case of an intra- and extra-cranial nasal venous malformation (VM) and bilateral internal jugular vein stenosis (BIJVS) in a 5-month-old-girl. Because of the BIJVS, the nasal VM served as primary venous outflow system for the central nervous system and therefore could not be managed with ablation. At six years of age, spontaneous recanalization of the bilateral internal jugular veins was documented on CT angiogram possibly expanding treatment options. To better understand and manage this unusual sequence of events, we performed a systematic literature review to identify similar cases and treatment. Methods A systematic literature review was conducted to identify the incidence of spontaneous recanalization of the jugular venous system. Six databases were utilized (PubMed, Google Scholar, Medline, Scopus, Cochrane, and CINAHL Complete) with the search terms “internal jugular vein stenosis” and “spontaneous recanalization and jugular vein,” and 101 citations were obtained for the literature review. These articles were manually reviewed to remove duplicates, cases where recanalization was achieved through interventional methods, and irrelevant articles. Results The level-of-evidence from the literature review was relatively low. The only two articles included in the literature review were case reports of spontaneous recanalization of thrombotic events, and only one included the internal jugular vein (IJV). The resulting low-evidence of our systematic literature search demonstrated the rarity of these unusual sequence of events. These articles recommend treatment, such as low-molecular weight heparin or endovascular balloons, in symptomatic pediatric patients with venous thrombosis; favorable outcomes were achieved regardless of treatment strategies. Discussion Spontaneous recanalization of our pediatric patient’s IJVS allows for multiple new treatment options of her nasal VM. However, before any treatment of the nasal VM is performed, adequate blood flow through the IJVs must be assured. Without complete recanalization and sufficient blood flow through the IJVs, ablation of the nasal VM may leave the central nervous system with decreased venous outflow leading to increased intracranial pressure. After determination of the flow sufficiency through the IJV, surgical excision of the nasal VM may be possible. If flow through the IJV is not sufficient, balloon angioplasty, patch angioplasty, or possible reconstruction of the IJVs may be necessary to allow for treatment of the nasal VM. If these surgical interventions prove to be too high-risk for the patient, conservative therapy may be the best option.

Comments

Mentor: Frank Lau (Department of Surgery, Plastics & Reconstructive Surgery)

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Oct 10th, 10:00 AM Oct 10th, 12:00 PM

Nasal Venous Malformation with Intracranial Communication: Presentation, Management, and Review of the Literature

Medical Education Building, LSUHSC-NO

Introduction We report a rare case of an intra- and extra-cranial nasal venous malformation (VM) and bilateral internal jugular vein stenosis (BIJVS) in a 5-month-old-girl. Because of the BIJVS, the nasal VM served as primary venous outflow system for the central nervous system and therefore could not be managed with ablation. At six years of age, spontaneous recanalization of the bilateral internal jugular veins was documented on CT angiogram possibly expanding treatment options. To better understand and manage this unusual sequence of events, we performed a systematic literature review to identify similar cases and treatment. Methods A systematic literature review was conducted to identify the incidence of spontaneous recanalization of the jugular venous system. Six databases were utilized (PubMed, Google Scholar, Medline, Scopus, Cochrane, and CINAHL Complete) with the search terms “internal jugular vein stenosis” and “spontaneous recanalization and jugular vein,” and 101 citations were obtained for the literature review. These articles were manually reviewed to remove duplicates, cases where recanalization was achieved through interventional methods, and irrelevant articles. Results The level-of-evidence from the literature review was relatively low. The only two articles included in the literature review were case reports of spontaneous recanalization of thrombotic events, and only one included the internal jugular vein (IJV). The resulting low-evidence of our systematic literature search demonstrated the rarity of these unusual sequence of events. These articles recommend treatment, such as low-molecular weight heparin or endovascular balloons, in symptomatic pediatric patients with venous thrombosis; favorable outcomes were achieved regardless of treatment strategies. Discussion Spontaneous recanalization of our pediatric patient’s IJVS allows for multiple new treatment options of her nasal VM. However, before any treatment of the nasal VM is performed, adequate blood flow through the IJVs must be assured. Without complete recanalization and sufficient blood flow through the IJVs, ablation of the nasal VM may leave the central nervous system with decreased venous outflow leading to increased intracranial pressure. After determination of the flow sufficiency through the IJV, surgical excision of the nasal VM may be possible. If flow through the IJV is not sufficient, balloon angioplasty, patch angioplasty, or possible reconstruction of the IJVs may be necessary to allow for treatment of the nasal VM. If these surgical interventions prove to be too high-risk for the patient, conservative therapy may be the best option.