Usefulness of endoscopic endonasal underwater surgery using a combination of coblation and a lens-cleaning system for hemostasis in hereditary hemorrhagic telangiectasia.

Hemostatic procedures for controlling nasal bleeding in refractory diseases such as hereditary hemorrhagic telangiectasia (HHT) can be challenging. In this report, we present a novel technique for underwater endoscopic endonasal hemostatic surgery, which was performed on a 69-year-old man with HHT. The patient had been experiencing frequent episodes of nasal bleeding and had many telangiectasias in the nasal cavity, which were the cause of the bleeding. These telangiectasias were effectively treated using a coblation device in combination with an endoscope lens-cleaning system that supplied saline to create stable underwater conditions. There are several advantages to this technique, including provision of a stable and clear endoscopic field of view, allowing for better visualization of the surgical site. This makes it easier to identify bleeding points and ensure accurate hemostasis. Additionally, the hydrostatic pressure created by the underwater environment helps to reduce bleeding during the procedure. However, it is important to take careful precautions to prevent water from entering the lower airway. With this precautionary measure, this technique is particularly useful in managing bleeding in patients with HHT.

Stereotactic radiosurgery for brain arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia.

OBJECTIVE: Brain arteriovenous malformations (AVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) present different characteristics from sporadic AVMs, and they have lower initial bleeding rates. Conservative management is usually preferred for the treatment of these lesions. In this case study, we present the largest series of HHT patients treated with stereotactic radiosurgery to date. METHODS: We identified eight patients with HHT and 14 AVMs. We retrospectively collected clinical, radiographic, and treatment characteristics of the patients and each AVM. RESULTS: Most patients in our sample presented with small AVMs. The median volume of these AVMs was 0.22 cm3 (IQR 0.08-0.59). Three out of eight patients presented with initial intracerebral hemorrhage (ICH). The majority of lesions had low (12/14) Spetzler-Martin grades (I-II). Median maximum and margin doses used for treatment were 36.2 (IQR 35.25-44.4) and 20 (IQR 18-22.5) Gy, respectively. The overall obliteration rate after SRS was 11/14, and the median time to obliteration across all 11 obliterated AVMs was 35.83 months (IQR, 17-39.99). Neurological status was favorable with all patients having a mRS of 0 or 1 at the last follow-up. Symptomatic radiation-induced changes (RIC) after SRS were low (7.1%), and there were no permanent RIC. CONCLUSIONS: Patients with HHT who present with multiple brain AVMs are generally well served by SRS. Obliteration can be achieved in the majority of HHT patients and with a low complication rate. In the current study, initial hemorrhage rates prior to SRS were noticeable which supports the decision to treat these AVMs. Future studies are needed to better address the role of SRS for HHT patients harboring ruptured and unruptured AVMs.

Postoperative Pain Following Coblation of Sinonasal Hereditary Hemorrhagic Telangiectasias.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare, autosomal dominant disease and epistaxis is the most common symptom. This can be treated conservatively but severe cases may require operative interventions. Endoscopic endonasal coblation of HHT lesions has been used successfully but postoperative pain management has not been well described. OBJECTIVES: This study aimed to assess levels of postoperative pain and opioid use among patients with HHT who underwent coblation of sinonasal lesions. METHODS: This is a longitudinal, prospective cohort study of adult patients undergoing endoscopic endonasal coblation for treatment of HHT lesions with or without bevacizumab injection between November 2019 and March 2020 at a single academic university hospital. Patients were given preoperative questionnaires and contacted via telephone 48 hours after surgery. If they reported using opioids for pain control, they were called every 2 days until they no longer used these medications. RESULTS: Fourteen cases, including 13 unique patients, were included in this study. Opioids were ordered on discharge in 4 cases and the average morphine milligram equivalent prescribed on discharge was 41. The median pain score on postoperative day (POD) 2 was 4 of 10. Twelve patients reported using acetaminophen and 4 were using opioid pain medications. Of those using opioid pain medications, only 1 patient was using opioid pain medication by POD 4 and denied any use after POD 10. CONCLUSION: This study is the first to analyze postoperative pain management and opioid prescribing patterns in HHT patients undergoing endonasal coblation of telangiectasias. Postoperative pain was mild to moderate and most patients stopped using opioid medications by POD 4, although the majority of patients solely used acetaminophen. Future studies with increased sample size will be useful to further identify predictors of need for analgesics postoperatively and other non-opioid adjuncts for pain control.

Lobectomy for Pulmonary Arteriovenous Fistula in a Patient With Rendu-Osler-Weber Disease: A Case Report.

BACKGROUND: Rendu-Osler-Weber disease (Osler's disease) is the most common cause of pulmonary arteriovenous fistula. We report a case of pulmonary arteriovenous fistula associated with Osler's disease that was treated by lobectomy. CASE REPORT: A 44-year-old man with Osler's disease presented with respiratory distress. Computed tomography showed a pulmonary arteriovenous fistula, which had a 26-mm-long diameter in S6 of the left lung. Transcatheter treatment had a high risk of recurrence, and surgery was indicated. The pulmonary arteriovenous fistula was found at the beginning of A6. A6 and the basilar artery were cut together with a stapler. The postoperative course was uneventful, and respiratory distress symptoms improved with no recurrence. CONCLUSION: We report a case of pulmonary arteriovenous fistula caused by Osler's disease treated by lobectomy. Although transcatheter treatment is the mainstream treatment for pulmonary arteriovenous fistulas, surgical resection may be effective depending on the size of the lesion.

Cerebral arterio-venous malformations hemodynamics changes in hereditary hemorrhagic telangiectasia - case report.

BACKGROUND: Spontaneous obliteration of cerebral arteriovenous malformations (AVMs) is a rare phenomenon. Hereditary Hemorrhagic Telangiectasia (HHT) is a predisposal genetic condition for AVMs development in all organs. CASE ILLUSTRATION: We report the case of a 34 years old woman with HHT family history. After radiosurgical treatment of a symptomatic evolving cerebellar AVM, late control subtracted digital angiography (DSA) demonstrated the complete obliteration of this AVM but also spontaneous obliteration of 3 fronto-parietal AVMs without any hemorrhagic sign on MRI. CONCLUSION: To our knowledge, this is the first report of spontaneous obliteration of multiple and unruptured AVMs in a HTT case.

Randomized, controlled, double-blinded clinical trial of effect of bevacizumab injection in management of epistaxis in hereditary hemorrhagic telangiectasia patients undergoing surgical cauterization.

BACKGROUND: Given its role in the disease pathophysiology, inhibition of vascular endothelial growth factor (VEGF)-mediated angiogenesis has received attention as a potential strategy to reduce epistaxis associated with hereditary hemorrhagic telangiectasia (HHT). In this study we evaluated the efficacy of a submucosal injection of bevacizumab, a VEGF inhibitor, in reducing the severity of epistaxis and improving quality of life when given at the time of operative electrocautery. METHODS: This randomized, double-blinded, placebo-controlled trial was conducted at a single institution from 2014 to 2019. Patients scheduled to undergo operative bipolar electrocautery of nasal telangiectasias were randomized to receive a submucosal injection of saline or bevacizumab at time of surgery. Surveys to assess epistaxis severity and quality of life (QOL), including the Epistaxis Severity Score (ESS) and the 12-item Short Form (SF-12), were administered preoperatively and at 1, 2, 4, and 6 months postoperatively. The minimal clinically important difference (MCID) of the ESS instrument is reported to be 0.71. RESULTS: Of 39 patients enrolled, 37 (94.9%) completed the study. The saline group demonstrated a reduced ESS vs baseline at 1 (-1.2; p = 0.01) and 4 (-1.2; p = 0.05) months postprocedure. The bevacizumab group demonstrated a reduced ESS score vs baseline at 1 (-2.3; p < 0.001), 2 (-2.3; p < 0.001), 4 (-2.0; p = 0.003), and 6 (-1.3; p = 0.05) months postprocedure. The additive benefit of bevacizumab over saline exceeded the MCID at 1, 2, and 4 months, but the difference was not statistically significant. CONCLUSION: The addition of a single treatment of submucosal bevacizumab may be associated with additional clinically meaningful benefit for up to 4 months when compared with electrocautery alone.

Snake Technique in Osler's Disease-Associated Thoracoabdominal Aortic Aneurysm.

Osler's disease is an autosomal dominant disorder characterized by epistaxis, telangiectases, and arteriovenous malformations of the internal organs. Herein, we report the first published case of dissecting thoracoabdominal aortic aneurysm repair in a 66-year-old woman with hereditary hemorrhagic telangiectasia associated with ACVRL1 gene mutations. We maintained the activated clotting time around 400 seconds during cardiopulmonary bypass to avoid lethal hemorrhage from telangiectases or arteriovenous malformations. The Adamkiewicz artery could not be identified on imaging studies preoperatively. Therefore, we used the snake technique for intercostal revascularization, which was effective in preventing paraplegia.

Resolution of Precapillary Pulmonary Hypertension After Liver Transplantation for Hereditary Hemorrhagic Telangiectasia: Systematic Review and Case Report.

BACKGROUND: Pulmonary hypertension (PH) is a known complication of hereditary hemorrhagic telangiectasia (HHT) in patients with hepatic arteriovenous malformations (HAVM). Orthotopic liver transplantation (OLT) is a recognized treatment of HAVM in HHT, but its effect on PH has not been reported in detail before. METHODS: Systematic review on HHT patients with pre- or postcapillary PH who underwent OLT and report of a case. RESULTS: Twenty-one patients were included from 7 articles, all case reports or case series. All had high-output cardiac failure prior to OLT. Two patients had precapillary PH, both related to ALK1 mutations. All patients but 1 showed significant improvement or complete resolution of PH after transplantation. One patient died of acute cardiac failure postoperatively. We also report the case of a 72-year-old woman with type 3 HHT and severe mixed pre- and postcapillary PH. The patient presented with multiple HAVM, left-to-right shunting, and severe but partially reversible combined pre- and postcapillary PH, without ALK1 mutation. After recurrent cholangitis episodes, liver abscesses, and severe obstruction of the right-sided biliary tree, an interdisciplinary decision was taken to proceed with OLT despite PH. Intraoperatively, PH resolved almost instantly after hepatic artery ligation and hepatectomy. CONCLUSIONS: In our patient, OLT completely abrogated mixed pre- and postcapillary PH. Based on this systematic review, we suggest that OLT should be considered a viable treatment option in patients with HHT, HAVM, and mixed pre- and postcapillary PH, featuring cardiac failure and drug responsive PH, rather than being seen as a major risk factor for cardiopulmonary complications.

Cauterización transoral de la arteria maxilar interna en la fosa infratemporal: informe de un caso / Transoral cauterization of the internal maxillary artery in the infratemporal fossa: case report

Se describe el caso clínico de una paciente de 84 años que tuvo epistaxis recidivante por padecer enfermedad de Rendu-Osler-Weber. Tuvo antecedentes de diversos tratamientos quirúrgicos que incluyeron el cierre de la fosa nasal izquierda (operación de Young). Por la persistencia de epistaxis izquierda se indicó una angiografía y embolización. Esta última no se hizo porque se diagnosticaron anastomosis entre el sistema carotídeo externo y el interno. Se realizó un abordaje intraoral paramaxilar asistido con endoscopios para cauterizar la arteria maxilar interna en la fosa infratemporal y un abordaje externo para cauterizar la arteria etmoidal anterior solucionando la epistaxis. (AU)

The clinical case of an 84-year-old patient who had recurrent epistaxis due to Rendu-Osler- Weber disease is described. She had a history of various surgical treatments including closure of the left nostril (Young's operation).Due to the persistence of left epistaxis, angiography and embolization were indicated. The latter was not done because anastomosis between the external and internal carotid system was diagnosed. An intraoral paramaxillary approach assisted with endoscopes was performed to cauterize the internal maxillary artery in the infratemporal fossa and an external approach to cauterize the anterior ethmoidal artery solving the epistaxis. (AU)

Resection of Clustered Arteriovenous Malformations to Avoid Lung Transplantation.

A 54-year-old man with hereditary hemorrhagic telangiectasia and severe hypoxemia was referred for lung transplantation. Embolization had not been performed because of numerous bilateral small pulmonary arteriovenous malformations. Although he appeared to be qualified for lung transplantation, we instead performed bilateral thoracoscopic multiple wide wedge resections because of his age, lifestyle as a farmer, and relatively clustered distribution of arteriovenous malformations. Intermittent bilateral ventilation was needed because of poor oxygenation in the early stages of the operation, but his oxygenation improved as the resection progressed. His postoperative oxygenation improved significantly, and lung transplantation was avoided.

In-Office KTP Laser for Treating Hereditary Hemorrhagic Telangiectasia-Associated Epistaxis.

OBJECTIVE: To evaluated the efficacy and safety of in-office potassium titanyl phosphate (KTP) laser treatment for the management of epistaxis in hereditary hemorrhagic telangiectasia (HHT) patients. METHODS: A retrospective case series of all HHT patients over age of 18 who underwent in-office KTP laser treatment from July 1, 2017 to December 31, 2019 was performed. The primary outcome measure was the epistaxis severity score (ESS) pre- and post-procedure. Secondary outcome measures included patient reported pain (on a 10-point Likert-type scale), and procedural adverse events and complications. RESULTS: A total of 16 patients underwent KTP in-office laser treatment during the review period. There was both a clinically and statistically significant decrease in the ESS after in-office laser treatment, baseline ESS -7.24, SD 1.71, follow up ESS -4.92, SD 1.83 (mean difference 2.94, 95% confidence interval, 1.83-4.04, P < .0001). There were no reported adverse events or complications associated with the procedure. The mean pain score reported was 0.19, SD 0.75. The average blood loss was 10.8 mL, SD 37.3. The majority of patients (62.5%, 10/16) had no blood loss during the procedure. CONCLUSION: Clinically and statistically significant decreases were noted in the ESS of HHT patients after in-office KTP laser photocoagulation. The procedure was well tolerated by patients, without any adverse events or complications. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E689-E693, 2021.

Pulmonary arteriovenous malformation managed by VATS lobectomy.

Pulmonary arteriovenous malformation (PAVM) is formed by abnormal connections between pulmonary arteries and veins that bypass the pulmonary capillaries and transport deoxygenated blood through pulmonary veins to the left heart. This causes insufficient oxygenation of blood in the lungs. This condition remains symptomless for a long period of time. The most common symptoms include shortness of breath on exertion, nosebleeds, increased fatigue and a gradual development of cyanosis. Paradoxical embolism in the brain is a serious complication; it can present with a stroke or a brain abscess. Treatment of the disease consists of embolization of the pathological vascular connections, surgical resection of the affected pulmonary parenchyma and management of concomitant manifestations of the disease. PAVM in most common cases arises as a result of an autosomal dominant hereditary disorder referred to as hereditary hemorrhagic telangiectasia.  Case report: In our communication, we document the diagnostic and therapeutic management in a young patient diagnosed with PAVM after falling off his bicycle. Based on comprehensive assessments, AV malformations with a 40% shunt of the pulmonary circulation were detected. An angiographic procedure was not an appropriate option considering the type and extent of the condition. Therefore, video-assisted thoracic resection of the affected pulmonary lobe was indicated. Conclusion: PAVM is a rare finding. PAVM should be ruled out in all patients with hereditary hemorrhagic telangiectasia (HHT) signs in the oral cavity. Contrast sonography of the heart and contract CT of the chest are the methods of choice for the diagnosis. Conservative or pharmacological treat-ment fails to improve the patients status.  The condition is usually managed by embolization. Cases where PAVM is rather extensive or diffuse, where endovascular management would be inappropriate, can be well managed using endoscopic resection adequate to the extent of the condition.

Treatment of tongue telangiectasia in a patient with hereditary haemorrhagic telangiectasia.

A 61-year-old Caucasian woman presented to an outpatient otolaryngology clinic with increased bleeding from a dorsal tongue telangiectasia for 3 weeks. Her history was significant for hereditary haemorrhagic telangiectasia (HHT), a rare condition that causes vascular dysplasia, and recent symptomatic anaemia requiring blood transfusions. After failing medical management with topical haemostatic agents, she was offered and underwent surgical intervention to remove the tongue telangiectasia with duel therapy potassium titanyl phosphate (KTP) laser coblation and bevacizumab injections. A team of otolaryngologists removed the lesion without complications, and the patient denied bleeding, had minimal pain, and endorsed increased quality of life postoperatively. Tongue telangiectasias can cause life-threatening bleeding in some patients with HHT, and no surgical management guidelines exist to treat them. This case demonstrates the efficacy of KTP laser followed by bevacizumab injections in treating tongue telangiectasias in a patient with HHT.

Hereditary Hemorrhagic Telangiectasia in a Sickle Cell Trait Patient: A Report of a Rare Case with Use of Nuclear Medicine, and a Literature Review.

BACKGROUND Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare autosomal dominant disease. CASE REPORT Here, we report a case of a 49-year-old Brazilian woman with a history of multiple hospitalizations, sometimes life-threatening anemia, and uncommon clinical manifestations. CONCLUSIONS We provide a brief literature review regarding the most common signs and symptoms, history, diagnosis, and treatment. Special attention is given to the techniques for identifying hemorrhagic areas, to the presence of angiodysplasia in gastric tissue, and the identification of sickle cell trait, this being an unprecedented hematological condition in the presentation of the disease. Thus, further studies on the relationship between sickle cell trait and the syndrome are needed.

Patient-recorded benefit from nasal closure in a Danish cohort of patients with hereditary haemorrhagic telangiectasia.

BACKGROUND: Nasal closure, also known as the modified Young's procedure was introduced in Denmark in 2008, as a surgical solution to severe epistaxis in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of this study was to report the overall satisfaction of the procedure from a patient's point of view as well as the occurrence of complications. METHODS: All the HHT patients who underwent nasal closure from 2008 to 2018 were included in the study. The patients were evaluated for postoperative complications and subjective outcome using Glasgow Benefit Inventory (GBI). RESULTS: Ten patients were included in the study and were observed for a mean of 64 months. None of the patients was completely free of complications, and reversal was requested in a single case. Haemoglobin levels rose with an average of 2.8 g/dl. The average GBI score after surgery was 38.05. Nine of ten patients would recommend nasal closure to fellow HHT patients. CONCLUSION: Nasal closure is highly recommended among patients, but due to the rate of postoperative complications, the procedure should be reserved for a carefully selected group of HHT patients.

[A Case of Ruptured Cerebral Arteriovenous Malformation Associated with Hereditary Hemorrhagic Telangiectasia].

BACKGROUND: Patients with hereditary hemorrhagic telangiectasia(HHT)are known to have high rates of cerebral arteriovenous malformations(AVMs). Compared to patients with sporadic AVMs, patients with HHT are less likely to present with ruptured AVMs. CASE REPORT: A 14-year-old male patient presented with headache that had lasted for 2 days. CT revealed an intracerebral hemorrhage in the right parietal lobe, and enhanced CT revealed an AVM in the upper part of the hematoma. The size of the nidus was 20 mm, and its feeders were the right superior internal parietal artery and a branch of the anterior cerebral artery. In addition, the AVM had no deep drainer. We also found another AVM in the right temporal lobe and identified telangiectasia of the nose using digital subtraction angiography. We suspected HHT and performed whole body CT, which revealed an arteriovenous fistula in the right lung and a hematoma-like lesion in the spleen. Thus, we diagnosed the patient with HHT. His ruptured AVM was removed electively. CONCLUSION: We report a case of HHT that presented as an intracerebral hemorrhage in a patient. Based on our case study findings, it is necessary to perform long-term follow-up not only for brain AVMs but also for visceral vascular malformations in such patients, as well as perform HHT screening for families. Although such cases are rare, some features of HHT must be considered to accurately diagnose suspected HHT.

Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States.

BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (1) Scientific Registry of Transplant Recipients (SRTR) database (1998-2016) (2) Single center liver transplant database (Mayo Clinic Rochester, MN). The aims of this study were (1) to determine trends in LT for HHT-related liver involvement in the United States using the SRTR database; (2) to identify clinical characteristics, indications, and outcomes for LT in HHT. RESULTS: Thirty-nine HHT patients were listed for LT in the SRTR database from 1998-2016 to 1998-2001 (n = 1); 2002-2005 (n = 4); 2006-2010 (n = 10), and 2011-2016 (n = 24). Twenty-four underwent LT at a median age of 47.5 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 7.0-9.5), and 75% received an exception MELD score. Two status-1 patients died during transplant surgery. Nineteen (86%) patients were alive after a median post-LT follow-up of 48 months, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, and 1 with biliary ischemia. All 5 were alive at the time of last follow-up with good graft function and resolution of heart failure. CONCLUSIONS: Outcomes after LT for HHT patients are excellent with 86% survival after a median follow-up of 48 months and resolution of heart failure. LT listing for HHT has increased in substantially in more recent eras.