CONTEXT:

The only treatment available to restore normal cardiac output in patients with hereditary hemorrhagic telangiectasia (HHT) and cardiac failure is liver transplant. Anti-vascular endothelial growth factor treatments such as bevacizumab may be an effective treatment.

OBJECTIVES:

To test the efficacy of bevacizumab in reducing high cardiac output in severe hepatic forms of HHT and to assess improvement in epistaxis duration and quality of life.

DESIGN, SETTING, AND PATIENTS:

Single-center, phase 2 trial with national recruitment from the French HHT Network. Patients were 18 to 70 years old and had confirmed HHT, severe liver involvement, and a high cardiac index related to HHT.

INTERVENTION:

Bevacizumab, 5 mg per kg, every 14 days for a total of 6 injections. The total duration of the treatment was 2.5 months; patients were followed up for 6 months after the beginning of the treatment.

MAIN OUTCOME MEASURE:

Decrease in cardiac output at 3 months after the first injection, evaluated by echocardiography.

RESULTS:

A total of 25 patients were included between March 2009 and November 2010. Of the 24 patients who had echocardiograms available for reread, there was a response in 20 of 24 patients with normalization of cardiac index (complete response [CR]) in 3 of 24, partial response (PR) in 17 of 24, and no response in 4 cases. Median cardiac index at beginning of the treatment was 5.05 L/min/m2 (range, 4.1-6.2) and significantly decreased at 3 months after the beginning of the treatment with a median cardiac index of 4.2 L/min/m2 (range, 2.9-5.2; P<001). Median cardiac index at 6 months was significantly lower than before treatment (4.1 L/min/m2; range, 3.0- 5.1). Among 23 patients with available data at 6 months, we observed CR in 5 cases, PR in 15 cases, and no response in 3 cases. Mean duration of epistaxis, which was 221 minutes per month (range, 0-947) at inclusion, had significantly decreased at 3 months (134 minutes; range, 0-656) and 6 months (43 minutes; range, 0-310) (P=.008). Quality of life had significantly improved. The most severe adverse events were 2 cases of grade 3 systemic hypertension, which were successfully treated.

CONCLUSION:

In this preliminary study of patients with HHT associated with severe hepatic vascular malformations and high cardiac output, administration of bevacizumab was associated with a decrease in cardiac output and reduced duration and number of episodes of epistaxis. (HEPATOLOGY 2013.).

BACKGROUND:

Oxymetazoline nasal spray is not FDA approved for use in children less than 6 years; however, its safety and efficacy are widely accepted, and it is in widespread use in children prior to procedures that may lead to epistaxis. We report a case of intraoperative oxymetazoline toxicity in a 4-year-old boy that led to a hypertensive crisis. While examining the possible causes for this problem, we became aware that the method of drug delivery led to an unanticipated overdose. The position in which the bottle is held causes pronounced variation in the quantity of oxymetazoline dispensed.

METHODS:

To examine the impact that bottle position has on the volume delivered, we measured the volume of oxymetazoline dispensed with the bottle in the upright and inverted position. We also measured the volume of a drop of oxymetazoline dispensed from the bottle. Because an additional source of oxymetazoline exposure is from packing the nares with surgical pledgets, we analyzed the volume of oxymetazoline absorbed by each pledget.

RESULTS:

Squeezing the bottle in the upright position results in a fine spray of fluid that averaged 28.9 ± 6.8 μl and was largely independent of effort. This volume is nearly identical to the measured volume of a drop of oxymetazoline, which was 30 μl. However, squeezing the bottle in the inverted position resulted in a steady stream of fluid, and the volume administered was completely effort dependent. Multiple tests in the inverted position demonstrated an average volume of 1037 ± 527 μl, with a range of 473-2196 μl. Lastly, the volume of oxymetazoline absorbed by each surgical pledget was 1511 ± 184 μl.

DISCUSSION:

Our testing indicates that bottle position during oxymetazoline administration can cause up to a 75-fold increase in intended drug administration.

OBJECTIVES:

We report our 5-year experience of continuous flow left ventricular assist device (LVAD) implantation without the use of anti-platelet therapy.

METHODS:

Between February 2006 and September 2011, 27 patients (26 men; 1 woman) were implanted with a continuous flow LVAD (HeartMate II, Thoratec Corporation, Pleasanton, CA, USA). The mean age was 55.7 ± 9.9 years. The mean duration of support was 479 ± 436 (1-1555) days with 35.4 patient-years on support. Twenty-one patients were implanted as a bridge to transplantation and 6 for destination therapy. The anticoagulation regimen was fluindione for all patients, with aspirin for only 4 patients. At the beginning of our experience, aspirin was administered to 4 patients for 6, 15, 60 and 460 days. Due to gastrointestinal (GI) bleeding and epistaxis, aspirin was discontinued, and since August 2006, no patients have received anti-platelet therapy.

RESULTS:

At 3 years, the survival rate during support was 76%. The most common postoperative adverse event was GI bleeding (19%) and epistaxis (30%) (median time: 26 days) for patients receiving fluindione and aspirin. The mean International Normalized Ratio (INR) was 2.58 ± 0.74 during support. Fifteen patients have been tested for acquired Von Willebrand disease. A diminished ratio of collagen-binding capacity and ristocetin cofactor activity to Von Willebrand factor antigen was observed in 7 patients. In the postoperative period, 2 patients presented with ischaemic stroke at 1 and 8 months. One of these 2 patients had a previous history of carotid stenosis with ischaemic stroke. There were no patients with haemorrhagic stroke, transient ischaemic attack or pump thrombosis. The event rate of stroke (ischaemic and haemorrhagic) per patient-year was 0.059 among the patients without aspirin with fluindione regimen only.

CONCLUSIONS:

A fluindione regimen without aspirin in long-duration LVAD support appears to not increase thromboembolic events and could lead to a diminished risk of haemorrhagic stroke.

OBJECTIVES

/

HYPOTHESIS:

When combined with local sphenopalatine (SP) injection and moderate hypotension, transoral or transcutaneous local injection of the anterior palatine (AP) vessels reduces intraoperative bleeding in hereditary hemorrhagic telangiectasia (HHT) patients undergoing nasal surgery.

STUDY DESIGN:

Retrospective chart review of 55 consecutive HHT patients undergoing a bevacizumab injection for recalcitrant epistaxis. Nineteen patients received local injections to only the SP vasculature, and 36 patients received AP and SP injections.

METHODS:

Main outcome variable was estimated blood loss during nasal surgery. Independent variables included sex, age, epistaxis severity score, surgical techniques (including laser), and blood pressure parameters (baseline, preinduction, and postintubation).

RESULTS:

The mean blood loss in HHT patients receiving SP injections alone was 111 mL, whereas it was 22 mL for those receiving both AP + SP injections. This difference between groups approached statistical significance (P = .075). Blood pressure parameters were similar in both groups with no appreciable difference between intraoperative systolic and mean arterial blood pressure.

CONCLUSIONS:

The addition of either sublabial or transcutaneous AP injection to the standard SP block markedly reduces blood loss in HHT epistaxis nasal surgery.

LEVEL OF EVIDENCE:

4. Laryngoscope, 2013.

To analyze the characteristics of patients who needed a blood transfusion due to epistaxis-caused anemia and to define potential risk factors.Retrospective cohort study.A total cohort of 591 epistaxis patients, prospectively included between March 2007 and April 2008 at the ENT department of the University Hospital of Zurich, was evaluated concerning the need for blood transfusions.The clinical charts and medical histories of these patients were evaluated.Common parameters that increase the risk for severe anemia due to epistaxis.Twenty-two patients required blood transfusions due to their medical condition. 22.7% suffered from traumatic nosebleeds. Another 27.3% had a known medical condition with an increased bleeding tendency. These proportions were significantly higher than in the group of patients without need of blood transfusion. The odds ratio for receiving a blood transfusion was 14.0 in patients with hematologic disorders, 4.3 in traumatic epistaxis and 7.7 in posterior bleeders. The transfusion-dependent epistaxis patients suffered significantly more often from severe posterior nosebleeds with the need for a surgical therapeutic approach.Patients with severe nosebleeds either from the posterior part of the nose or with known hematologic disorders or traumatic epistaxis should be closely monitored by blood parameter analyses to evaluate the indication for hemotransfusion. The acronym THREAT (Trauma, Hematologic disorder, and REAr origin of bleeding → Transfusion) helps to remember and identify the factors associated with an increased risk of receiving blood transfusion.

Papillary seromucinous adenocarcinoma of the sinonasal tract is exceedingly rare. The objectives of this case report are to describe a case of papillary seromucinous adenocarcinoma presenting in the nasopharynx and to review the literature pertaining to other similar cases.A review of the patient's chart and a review of the English literature were conducted.We describe the case of a 64 year-old woman who presented with a 3-year history of epistaxis and right-sided otitis media with effusion. The patient had been followed for a known nasopharyngeal mass that had twice been biopsied and in both cases was considered a benign mass pathologically. A third biopsy was diagnosed as a low-grade papillary seromucinous adenocarcinoma. The patient was otherwise asymptomatic. The patient was referred to a multidisciplinary cancer clinic at which endoscopic resection was determined to be the preferred treatment modality. A literature review and approach to patients with nasopharyngeal masses will be presented.Papillary seromucinous adenocarcinoma is a rare tumor that can present in the nasopharynx. We describe the endoscopic surgical management of one such patient that presented to our care.

To describe the clinico-pathologic manifestations of sinonasal malignancies, identify the contributing factors to delay in presentation and recommend ways of preventing them in a resource challenged environment.A questionnaire based cross sectional descriptive study of patients with sinonasal malignancies between 2006 and 2011.Hospital based study at the Otorhinolaryngology Department, University College Hospital, Ibadan. Participants: 61 patients diagnosed with sinonasal malignancies.Patients demographic and essential medical data were collected with a structured, interviewer assisted questionnaire and results analysed using descriptive statistics.There were 28(45.9%) males and 33(54.I %) females; mean age 37years. The common presentations were epistaxis, nasal obstruction and facial asymmetry and 96.7% patients with squamous cell carcinoma presented in advanced disease stage (Stage 3 & 4). Over 47% patients presented a year after onset of symptoms. Factors which included self-medication, wrong advice from relations/friends to consult traditional herbalist or quacks for treatment and traditional & religious beliefs contributed to delay in presentation to hospitals. High cost of medical treatment, unwelcoming attitudes of some hospital staff, lack of confidence in orthodox medicine and proximity to health facilities were reasons given for not considering hospital as the first place to seek medical treatment.Health education to create awareness of sinonasal malignancies and provision of affordable and accessible health facilities especially in rural areas are recommended ways to encourage patients to present early in hospitals. This will improve the management outcome and quality of life of patients with sinonasal malignancies.

OBJECTIVES

. To evaluate the efficacy and complications of endovascular treatment for ruptured internal carotid artery pseudoaneurysms following irradiation of nasopharyngeal carcinoma.

DESIGN.

Retrospective case series.

SETTING.

Tertiary neurosurgical referral unit of a Hong Kong public hospital.

PATIENTS.

Patients with ruptured radiation-induced internal carotid artery pseudoaneurysms that were treated endovascularly from October 1999 to October 2011 at Queen Elizabeth Hospital were reviewed. Hospital records, imaging, and angiographic data were studied.

RESULTS.

During the study period, 15 such nasopharyngeal carcinoma patients were treated by endovascular means at Queen Elizabeth Hospital. Ten presented with epistaxis, three with otorrhagia (bleeding from the ear), and two with both. Therapeutic occlusion of the affected internal carotid artery was performed in four patients, and stenting of the artery (with or without coil obliteration of the pseudoaneurysm) was performed in 11. Immediate haemostasis was achieved in all cases. One (7%) of the 15 patients endured symptomatic recurrence of the pseudoaneurysm, and in another an asymptomatic residual pseudoaneurysm was noted in the follow-up angiogram. Three patients suffered clinically significant procedure-related complications, including cerebral infarction (n=2) and brain abscess (n=1). In the angiograms obtained after a mean post-treatment interval of 13 (range, 0.7-60) months, the stent patency rate was 67%. All three patients with occluded stents were asymptomatic.

CONCLUSIONS.

Ruptured internal carotid artery pseudoaneurysms following radiotherapy is a rare but life-threatening condition. Endovascular treatment by occlusion or reconstruction of the internal carotid artery with stents provides immediate haemostasis and obliteration of the pseudoaneurysms, with a low recurrence rate. Long-term follow-up is necessary to look out for delayed post-treatment complications.