Uremic leontiasis ossea is a rare condition, reported in patients with severe renal disease. Patients present with progressive enlargement of facial bones-in particular, the maxillary and mandibular bones. Rarity of this condition leaves clinicians puzzled on initial evaluation and management. Herein, we present a 31year-old man diagnosed with uremic leontiasis ossea. The report aims to review the pathophysiology of the condition as described in the literature, the patient presentation and imaging modalities used to investigate, and classical findings seen in patients with uremic leontiasis ossea. Finally, we briefly touch base on the reported regimens used to prevent and manage this condition.
Background
Congenital portosystemic shunts are embryological malformations in which portal venous flow is diverted to the systemic circulation. High morbidity and mortality are seen in patients with concurrent hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension. Endovascular therapy, in the correct patient population, offers a less invasive method of treatment with rapid relief of symptoms.
Case presentation
In this report, we discuss the treatment of a two-year-old male with abnormal chorea-like movements, altered mental status, anisocoria and hyperammonemia diagnosed with an intrahepatic congenital portosystemic shunt between the inferior vena cava and right portal vein. Given the patient’s amenable anatomy and shunt type, embolization was performed with an 18 mm Amplatzer patent foramen ovale occlusion device.
Conclusions
Portosystemic shunts are a rare congenital abnormality without universal treatment guidelines. An Amplatzer PFO occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy.
Gastric varices are a sequela of portal hypertension. If left untreated, life-threatening bleeding can occur. While endoscopic treatment is traditionally considered as a first-line interventional option, endovascular techniques have emerged to become the superior option in certain clinical scenarios, either as a first-line therapy or a salvage therapy. Endovascular techniques for managing gastric varices include transjugular intrahepatic portosystemic shunt (TIPS) placement, balloon-occluded retrograde transvenous obliteration (BRTO), coil, and plug-assisted retrograde transvenous obliteration, as well as a combination of both. Studies have shown that endovascular techniques can effectively control bleeding from gastric varices, with high success rates and low complication rates, and significantly reduce recurrence. TIPS placement has shown to be more effective in controlling bleeding from gastroesophageal varices, while BRTO, coil, or plug-assisted embolizations are more suitable for patients with isolated gastric varices. Endovascular interventions are a valuable option for managing gastric varices, especially in patients who are not candidates for endoscopic treatment or who have failed previous endoscopic interventions. In this two-part series editorial, we aim to initially review the complex anatomy and classification of gastric varices, medical management, and current endovascular interventional techniques, and how they compare with one another. In part 2, we draw a parallel between endovascular versus endoscopic techniques, and highlight and critically review current literature as it pertains to gastric variceal management.
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