Higher prevalence of splenic artery aneurysms in hereditary hemorrhagic telangiectasia: Vascular implications and risk factors.

BACKGROUND: Splenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT. METHODS: We analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement. RESULTS: SAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03-4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02-1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01-4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF. CONCLUSIONS: We found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.

Pediatric sialendoscopy: a 5-year experience at a single institution.

OBJECTIVES: To evaluate the outcome of our experience in the treatment of salivary gland disorders in children undergoing sialendoscopy and to assess the evolution of the technique. DESIGN: Retrospective medical record review. SETTING: Tertiary care university hospital. PATIENTS: Thirty-eight children with salivary gland disorders undergoing diagnostic and interventional sialendoscopy between January 1, 2003, and November 30, 2008. INTERVENTION: Diagnostic and interventional sialendoscopy using general anesthesia. MAIN OUTCOME MEASURES: Demographic, clinical, and surgical variables, including age, sex, date of first symptoms, parotid or submandibular location of disease, preoperative ultrasonographic results, sialendoscopy technique, sialendoscopy observations, and complications. RESULTS: Pediatric sialendoscopy was performed on the parotid gland in 23 patients (61%) and on the submandibular gland in 15 patients (39%). The most frequent indication for sialendoscopy was recurrent salivary gland swelling. Thirty-two of 38 procedures (84%) were performed endoscopically, whereas a combined intervention was necessary for 3 patients and a submandibular gland excision for another 3 patients. Sialendoscopy allowed the diagnosis of 12 patients with salivary duct lithiasis, 21 with salivary duct stenosis, and 2 with both submandibular lithiasis and stenosis, and findings from 3 sialendoscopies were normal. Preoperative ultrasonographic results were confirmed by sialendoscopy in only 7 patients. Of the 10 patients with lithiasis found using sialendoscopy, only 4 had been detected using preoperative ultrasonography. CONCLUSIONS: Sialendoscopy is a pertinent technique for the diagnosis and treatment of salivary gland disorders in children. It also allows the most conservative treatment of sialolithiasis and juvenile recurrent parotitis.