A Rare Association: Hereditary Hemorrhagic Telangiectasia with Liver Cirrhosis Causing Portal Hypertension.

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a vascular disorder of autosomal dominant etiology. The hallmark clinical feature is the presence of recurrent episodes of epistaxis in patients with vascular malformations and a tendency to bleed. We present the case of a 71-year-old woman who presented to the emergency department with upper gastrointestinal bleeding caused by esophageal varices, in conjunction with gastric angiodysplasias. The presence of oronasopharyngeal telangiectasias and hepatomegaly raised suspicion of HHT. The diagnostic workup confirmed the presence of angiodysplasia in the gastric region, portal arteriovenous malformation, and a pulmonary shunt.

Descriptive Analysis of the Arterial Supply to the Auricle in Patients with Unilateral Microtia.

BACKGROUND: Microtia is a congenital auricular deformity that occurs in 1:5,000-10,000 births. It can cause severe impairment to the patient's self-esteem and problems regarding social integration. Multiple measures have been described in attempt to better operative outcomes of these patients. We used computed tomography (CT) angiography to analyze the vascular pattern of the auricular region before surgery. METHODS: Fourteen patients with unilateral microtia were included. All underwent CT angiogram plus tridimensional reconstruction. Both healthy and microtic auricles were analyzed descriptively in terms of main arterial supply, pattern, diameter of subbranches, and angulation. The sample was divided in 2 age groups for better understanding of the data. RESULTS: Blood supply to the auricle was found to depend on 2 main vessels: temporal superficial artery (TSA) and its subbranches (superior, middle, and lower branch) and posterior auricular (PA) artery. In the microtic group, TSA was the dominant artery in 13 of 14 cases (92%). Superior, middle, and inferior branches were present in 4, 3, and 0 cases, respectively. Three of the microtic auricles presented supply from PA artery, from which in 1 case, it represented the only supply to the region. CONCLUSIONS: There is wide variability in the blood supply of both healthy and microtic auricles; however, we were able to identify some tendencies in our sample. Further research is needed to prove the benefit of a preoperative imaging study in these patients. Still, in our experience, we found it useful as a complement for surgical planning.