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BACKGROUND: Exercise-induced pulmonary haemorrhage (EIPH) in athletic horses is characterized by the presence of blood from the lungs in the tracheobronchial tree after intense exercise. Despite the high prevalence of EIPH in horses, the primary aetiology remains unknown. Variants in the genes encoding CD39 and CD39L1 (ENTPD1 and ENTPD2, respectively) were previously reported as potential genetic causes involved in EIPH pathogenesis. However, the role of these variants in haemostatic functions is unknown. RESULTS: To investigate the association between EIPH and missense variants in the ENTPD1 (rs1152296272, rs68621348, and rs68621347) and ENTPD2 genes (rs782872967), 76 Thoroughbred horses diagnosed with EIPH and 56 without clinical signs of EIPH (control group) by trachea-bronchial endoscopy were genotyped. The rs1152296272 and rs68621347 variants were linked, which explained why the same results were found in all horses. Approximately 96% and 95% of the EIPH and control horses, respectively, carried at least one nonreference allele for these variants. In contrast, 100% of the control horses and 96% of the EIPH horses were homozygous for the reference allele for the rs68621348 variant. In the EIPH group, 1.5% of the horses were homozygotes and 24% were heterozygous for the nonreference allele of the rs782872967 variant. In the control group, the nonreference allele of this variant was observed only in heterozygotes (16%). There were no significant differences between groups for any of the variants. CONCLUSIONS: The variants previously described in the genes encoding the CD39 and CD39L1 enzymes were highly present in the studied population. However, no association was found between the occurrence of EIPH and the presence of these variants in Thoroughbred horses in this study.

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Trastuzumab emtansine (T-DM1) is a targeted therapy combining trastuzumab and emtansine for human epidermal growth factor receptor 2(HER2)-positive breast cancer, with common side effects including fatigue, nausea, pain, headache, low platelet count, and elevated liver enzymes. Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant vascular dysplasia characterized by vascular malformations and telangiectasias in various organs. We present a case of a female patient with advanced breast cancer who developed HHT-like symptoms while on T-DM1 treatment. A 59-year-old woman treated with radiotherapy and T-DM1 every 21 days developed recurring nosebleeds and mucocutaneous and liver telangiectasias indistinguishable from HHT three months after receiving the first dose of T-DM1. Other organ vascular malformations were ruled out through screening protocols. The patient had no previous HHT symptoms or family history. Nasal care measures like lubrication and antifibrinolytics (tranexamic acid) were provided. In addition, propranolol was also prescribed due to its antiangiogenic and antitumoral properties, leading to significantly decreased epistaxis and telangiectasias. Microtubule disruptions caused by T-DM1, along with other angiogenic mechanisms may contribute to the development of telangiectasias resembling HHT. The use of propranolol, an initial approach for HHT, proved to be effective in this case. It is crucial for oncologists and HHT specialists to be aware of this rare adverse event associated with T-DM1 and to implement appropriate management strategies.

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OBJECTIVE: To analyze variability in the distance between the Anterior Ethmoidal Artery (AEA) and the anterior Skull Base (SB), as well as the frequency of lateral asymmetry in a Latin American population using computed tomography. METHODS: A total of 250 computed tomography scans of paranasal sinuses in coronal reconstruction (500 AEAs) were analyzed. After determining the image with the best anatomical view of the artery, the distance between its midpoint and the ethmoidal roof was measured, and the images were independently interpreted by 2 physicians. RESULTS: Of the 500 AEAs, 279 (55.8%) adhered to or passed through the SB at a distance of 0mm. A total of 221 AEAs (44.2%) were at some distance from the SB, of which 107 (48.4%) were on the right side, ranging from 1.18 to 6.75mm, and 114 (51.5%) were on the left side, ranging from 1.15 to 6.04mm. The overall mean distance between the AEA and SB was 1.22 (SD=1.57) mm, increasing to 2.77 (SD=1.14) when the arteries adhered to the SB were excluded. Seventy-six individuals (30.4%) had a lateral distance variation > 1mm. CONCLUSION: Our study includes the largest sample of AEA analyzed with computed tomography scans of paranasal sinuses. There was some distance between the AEA and SB in almost half the patients, and we found a high rate of lateral variability >1mm. LEVEL OF EVIDENCE: Level 3.

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Abstract Objective To analyze variability in the distance between the Anterior Ethmoidal Artery (AEA) and the anterior Skull Base (SB), as well as the frequency of lateral asymmetry in a Latin American population using computed tomography. Methods A total of 250 computed tomography scans of paranasal sinuses in coronal reconstruction (500 AEAs) were analyzed. After determining the image with the best anatomical view of the artery, the distance between its midpoint and the ethmoidal roof was measured, and the images were independently interpreted by 2 physicians. Results Of the 500 AEAs, 279 (55.8%) adhered to or passed through the SB at a distance of 0 mm. A total of 221 AEAs (44.2%) were at some distance from the SB, of which 107 (48.4%) were on the right side, ranging from 1.18 to 6.75 mm, and 114 (51.5%) were on the left side, ranging from 1.15 to 6.04 mm. The overall mean distance between the AEA and SB was 1.22 (SD = 1.57) mm, increasing to 2.77 (SD = 1.14) when the arteries adhered to the SB were excluded. Seventy-six individuals (30.4%) had a lateral distance variation > 1 mm. Conclusion Our study includes the largest sample of AEA analyzed with computed tomography scans of paranasal sinuses. There was some distance between the AEA and SB in almost half the patients, and we found a high rate of lateral variability >1 mm. Level of evidence: Level 3.

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OBJECTIVE: To assess the utility of the Curaçao criteria by age over time in children with hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN: This was a single-center, retrospective analysis of patients attending the HHT clinic at the Hospital for Sick Children (Toronto, Canada) between 2000 and 2019. The evaluation of the Curaçao criteria was completed during initial and follow-up visits. Screening for pulmonary and brain arteriovenous malformations was completed at 5 yearly intervals. RESULTS: A total of 116 patients with genetic confirmation of HHT were included in the analysis. At initial screening at a median (IQR) age of 8.4 (2.8, 12.9) years, 41% met criteria for a definite clinical diagnosis (≥3 criteria). In children <6 years at presentation, only 23% fulfilled at least 3 criteria initially. In longitudinal follow-up, 63% reached a definite clinical diagnosis, with a median (IQR) follow-up duration of 5.2 (3.2, 7.9) years (P = .005). Specifically, more patients met the epistaxis and telangiectasia criteria at last visit compared with initial (79% vs 60%; P = .006; 47% vs 30%; P = .02) but not for the arteriovenous malformation criterion (59% vs 57%; P = .65). CONCLUSIONS: In the pediatric population, most patients do not meet definite clinical criteria of HHT at initial presentation. Although the number of diagnostic criteria met increased over time, mainly due to new onset of epistaxis and telangiectasia, accuracy remained low during follow-up visits. Relying solely on clinical criteria may lead to underdiagnosis of HHT in children.

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PURPOSE: The Curaçao criteria are well-established diagnostic criteria for hereditary hemorrhagic telangiectasia (HHT), but they lack details regarding a predictive presentation of epistaxis and telangiectasias. This study collects and compares data in HHT and population cohorts to inform the application of these criteria. METHODS: In-person interviews regarding epistaxis and targeted examination for telangiectases in a general population cohort (n = 204) and an HHT cohort (n = 432) were conducted. RESULTS: Frequency of epistaxis, rather than intensity or duration, was the best discriminator of HHT. A cutoff of ≥4 nosebleeds per year alone yielded a diagnostic sensitivity of 97%, and specificity of 84%. The mean number of telangiectases at the sites investigated was 0.4 in the general population cohort and 26.5 in the HHT cohort. The most distinctive sites for telangiectases in HHT were lips and palmar fingers, whereas telangiectases of the face and dorsum of the hand were comparable in both cohorts. CONCLUSION: We propose that the Curaçao criteria be modified to include the following cutoffs: (1) epistaxis frequency of ≥4 nosebleeds per year and (2) telangiectasia count of at least 2 in characteristic locations (palmar aspect of fingers, lips, and oral cavity), and that cutaneous telangiectases at other sites not be considered relevant for diagnostic purposes.

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Glomangiopericytoma is a rare vascular neoplasm of the nasal cavity and paranasal sinuses that occurs during the sixth or seventh decade of life. It is categorized as a borderline tumor with low malignant potential and classified as a distinct entity of sinonasal tumors with perivascular myoid phenotype by the World Health Organization (WHO). We report the case of a 50-year-old woman with nasal obstruction and severe epistaxis. The nasal sinuses computed tomography (CT), and magnetic resonance imaging (MRI) demonstrated a 3.1 cm soft tissue mass occupying the upper part of the left nasal cavity invading the left paranasal sinuses and nasal septum, and the left eye medial rectus muscle. A total mass resection was performed by nasal endoscopy. The histological and immunohistochemical examination yielded the diagnosis of glomangiopericytoma. This case report aims to contribute to the knowledge of nasal neoplasms. The need for more data on this entity is the main obstacle to developing standardized treatment guidelines.

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El manejo del sangrado en CEF es fundamental, ya que aumenta la tasa de complicaciones. El principal objetivo del manejo perioperatorio es lograr una buena hemostasia, es por esto que se realizó una revisión de las principales recomendaciones de la literatura internacional, que incluye medidas preoperatorias, intraoperatorias y postoperatorias.

Bleeding is an important factor in FESS since it increases the rate of complications. The main objective of perioperative management is to achieve good hemostasis. This is a review of the main recommendations of the international literature, that includes preoperative, intraoperative and postoperative measures.

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ABSTRACT Glomangiopericytoma is a rare vascular neoplasm of the nasal cavity and paranasal sinuses that occurs during the sixth or seventh decade of life. It is categorized as a borderline tumor with low malignant potential and classified as a distinct entity of sinonasal tumors with perivascular myoid phenotype by the World Health Organization (WHO). We report the case of a 50-year-old woman with nasal obstruction and severe epistaxis. The nasal sinuses computed tomography (CT), and magnetic resonance imaging (MRI) demonstrated a 3.1 cm soft tissue mass occupying the upper part of the left nasal cavity invading the left paranasal sinuses and nasal septum, and the left eye medial rectus muscle. A total mass resection was performed by nasal endoscopy. The histological and immunohistochemical examination yielded the diagnosis of glomangiopericytoma. This case report aims to contribute to the knowledge of nasal neoplasms. The need for more data on this entity is the main obstacle to developing standardized treatment guidelines.

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La telangiectasia hemorrágica hereditaria (THH) es una displasia vascular multisistémica, de herencia autosómica dominante, caracterizada por el desarrollo de telangiectasias mucocutáneas y malformaciones arteriovenosas viscerales. El diagnóstico se realiza mediante los criterios de Curasao publicados en el año 2000 y su manejo requiere de un equipo multidisciplinario donde el rol del otorrinolaringólogo(a) es fundamental, puesto que la epistaxis se presenta en un 90%-95% de los pacientes siendo una de las primeras manifestaciones clínicas de la enfermedad y pudiendo amenazar la vida del paciente. En la literatura se describen múltiples alternativas de tratamiento médico y quirúrgico para la epistaxis, sin existir un tratamiento definitivo para la enfermedad. A continuación, presentaremos el caso de una paciente de 56 años con THH y epistaxis recurrentes severas que, tras no responder al tratamiento médico conservador y múltiples procedimientos quirúrgicos, se realizó el cierre nasal mediante el procedimiento de Young, constituyendo el primer caso reportado en nuestro país.

Hereditary hemorrhagic telangiectasia (HHT) is a multisystemic vascular dysplasia, of autosomal dominant inheritance, characterized by the development of mucocutaneous telangiectasias and visceral arteriovenous malformations. The diagnosis is made using the Curafao criteria published in 2000 and its management requires a multidisciplinary team where the role of the ENTs is fundamental, since epistaxis occurs in 90%-95% of patients, being one of the first clinical manifestations of the disease and may threaten the life of the patient. Multiple medical and surgical treatment alternatives for epistaxis are described in the literature without a definitive treatment for the disease. Here, we present the case of a 56-year-old patient with severe recurrent HHT and epistaxis who, after not responding to the initial conservative and surgical treatments, a nasal closure was performed, using the Young's procedure, constituting the first case reported in our country.

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