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Spontaneously improving occipitocondylar hyperplasia: a case report.

ABSTRACT: This case report presents a pediatric patient with the extremely rare craniovertebral junction anomaly of occipitocondylar hyperplasia. This 4-year old boy presented with macrocephaly and mild to moderate pontomedullary compression from the unilateral occipitocondylar hyperplasia. Based on the asymptomatic clinical presentation, it was decided to follow the patient with serial magnetic resonance images without surgical intervention. Upon further imaging the occipitocondylar hyperplasia spontaneously resolved. This case report offers watchful waiting as an alternative treatment approach to surgical intervention as was reported in the literature previously. Possible pathophysiologic mechanisms are also briefly explored.

SUBMITTER: Nagy L

PROVIDER: S-EPMC4110150 | biostudies-other | 2014 Aug

REPOSITORIES: biostudies-other

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Project description:BackgroundHeyde's syndrome can be easily overlooked or misjudged in clinical practice because it shares common clinical manifestations with multiple diseases as well as limited accuracy of several corresponding examinations for diagnosing Heyde's triad. Moreover, aortic valve replacement is often delayed in these patients due to the contradiction between anticoagulation and hemostasis. Herein, we present a rare case of atypical Heyde's syndrome. The patient's severe intermittent gastrointestinal bleeding was not completely cured even through a local enterectomy. In the absence of direct evidence of acquired von Willebrand syndrome (AVWS) or angiodysplasia, her long-standing gastrointestinal bleeding was finally stopped after receiving transcatheter aortic valve implantation (TAVI).Case presentationA 64-year-old female suffered from refractory gastrointestinal bleeding and exertional dyspnoea. A local enterectomy was performed owing to persistent hemorrhage and repeated transfusions; subsequently, histological examination revealed angiodysplasia. Heyde's syndrome was not suspected until 3 years later, at which time the patient started bleeding again and was also found to have severe aortic valve stenosis upon echocardiography. TAVI was consequently performed when the patient was in a relatively stable condition even though the predisposition to bleed, but there was no evidence of angiodysplasia and AVWS during angiography at that time. The patient's above symptoms were significantly relieved after TAVI and followed up for 2 years without any significant ischemic or bleeding events.ConclusionsThe visible characteristics of angiodysplasia or a shortage of HMWM-vWFs should not be indispensable for the clinical diagnosis of Heyde's syndrome. Enterectomy could be a bridging therapy for aortic valve replacement in patients with severe hemorrhage, and TAVI may be beneficial for moderate to high surgical-risk patients even if they have a potential risk of bleeding.

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Spontaneously improving occipitocondylar hyperplasia: a case report.

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