scholarly journals Various patterns of the middle cerebral vein and preservation of venous drainage during the anterior transpetrosal approach

2016 ◽  
Vol 124 (2) ◽  
pp. 432-439 ◽  
Author(s):  
Shunsuke Shibao ◽  
Masahiro Toda ◽  
Maaya Orii ◽  
Hirokazu Fujiwara ◽  
Kazunari Yoshida
Keyword(s):  
Venous Drainage ◽  
Anatomical Variations ◽  
Cerebral Vein ◽  
Drainage Patterns ◽  
Anterior Transpetrosal Approach ◽  
Superficial Middle Cerebral Vein ◽  
Anterior Petrosectomy ◽  
Type 3

OBJECT The drainage of the superficial middle cerebral vein (SMCV) has previously been classified into 4 subtypes. Extradural procedures and dural incisions during the anterior transpetrosal approach (ATPA) may interrupt the route of drainage from the SMCV. In this study, the authors examined the relationship between anatomical variations in the SMCV and the corresponding surgical modifications to the ATPA that are necessary for venous preservation. METHODS This study included 48 patients treated via the ATPA in whom the SMCV was examined using 3D CT venography. The drainage patterns of the SMCV were classified into 3 types: cavernous or absent (Type 1), sphenobasal (Type 2), and sphenopetrosal (Type 3). Type 2 was subdivided into medial (Type 2a) and lateral (Type 2b), and Type 3 was subdivided into vein (Type 3a), vein and sinus (Type 3b), and sinus (Type 3c). The authors performed 3 ATPA modifications to preserve the SMCV: epidural anterior petrosectomy with subdural visualization of the sphenobasal vein (SBV), modification of the dural incision, and subdural anterior petrosectomy. Standard ATPA can be performed with Type 1, Type 2a, and Type 3a drainage. With Type 2b drainage, an epidural anterior petrosectomy with subdural SBV visualization is appropriate. The dural incision should be modified in Type 3b. With Type 3c, a subdural anterior petrosectomy is required. RESULTS The frequency of each type was 68.7% (33/48) in Type 1, 8.3% (4/48) in Type 2a, 4.2% (2/48) in Type 2b, 14.6% (7/48) in Type 3a, 2.1% (1/48) in Type 3b, and 2.1% (1/48) in Type 3c. No venous complications were found. CONCLUSIONS The authors propose an SMCV modified classification based on ATPA modifications required for venous preservation.

Angiographic Changes in Venous Drainage of Cavernous Sinus Dural Arteriovenous Fistulae after Palliative Transarterial Embolization or Observational Management: A Proposed Stage Classification

Neurosurgery ◽  
2005 ◽  
Vol 56 (3) ◽  
pp. 494-502 ◽  
Author(s):  
Junichiro Satomi ◽  
Koichi Satoh ◽  
Shunji Matsubara ◽  
Norio Nakajima ◽  
Shinji Nagahiro
Keyword(s):  
Cavernous Sinus ◽  
Transarterial Embolization ◽  
Venous Drainage ◽  
Arteriovenous Fistulae ◽  
Complete Cure ◽  
Drainage Patterns ◽  
Type 3

Abstract OBJECTIVE: We assessed whether angiographic changes in venous drainage patterns occur over time in cavernous sinus dural arteriovenous fistulae (dAVFs) without a complete cure. METHODS: We classified 65 cavernous sinus dAVFs into three types on the basis of initial angiographic findings. In Type 1, both anterior and posterior drainage routes were open; in Type 2, the posterior drainage route was closed, whereas the anterior drainage route was open; and in Type 3, both the posterior and anterior drainage routes were closed. RESULTS: Of the 65 dAVFs, 40 were of Type 1, 21 of Type 2, and 4 of Type 3. During the follow-up period, 17 of the dAVFs that were treated palliatively with transarterial embolization (n = 11) or monitored without therapy (n = 6) demonstrated angiographic changes. In these 11 patients, there was a change in the venous drainage pattern from Type 1 to Type 2 (n = 5), from Type 2 to Type 3 (n = 3), and from Type 1 to Type 3 (n = 3). One of 11 had a conversion into a lesion with cortical venous drainage. The remaining 6 dAVFs (4 with observational management, 2 with transarterial embolization) demonstrated closure of the fistula; in 5 of these, the affected cavernous sinus was not depicted on follow-up angiograms. CONCLUSION: In some cavernous sinus dAVFs with palliative transarterial embolization or observational management, there was a change in the venous drainage patterns, consisting of a decrease in the number of venous drainage routes. There was a trend for the posterior route to close before the anterior drainage or cortical drainage route. This suggests the occurrence of a staged progression in a regular direction in cavernous sinus dAVFs. Without treatment aiming at a complete cure, most cavernous sinus dAVFs can behave benignly, with a low possibility of development of cortical venous reflux during follow-up.

scholarly journals Association between the Anatomy of the Mandibular Canal and Facial Types: A Cone-Beam Computed Tomography Analysis

2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Rudyard dos Santos Oliveira ◽  
Arlete Maria Gomes Oliveira ◽  
José Luiz Cintra Junqueira ◽  
Francine Kühl Panzarella
Keyword(s):  
Anatomical Variations ◽  
Mandibular Canal ◽  
Root Tips ◽  
Morphological Variations ◽  
The Face ◽  
Facial Types ◽  
Type 3 ◽  
Facial Type

We evaluated the anatomical variations of the mandibular canal associated with various facial types, age, sex, and side of the face studied. We analyzed 348 hemimandibles in subjects without a history of trauma, lesions in the lower arch, or orthognathic or repair surgery in the posterior mandible. Facial type was determined using the VERT index. The canal path was classified as Type 1 (a large, single structure passing very close to the root tips); Type 2 (a canal passing closest to the mandibular base); and Type 3 (a canal present in the posterior mandibular region, with a lower canal running through the mandibular branch, reaching the anterior region). Bifid canals (type 3) were classified into four categories according to the course and number of mandibular canals. The brachyfacial and mesofacial types presented a Type 1 canal in 95.5% (n=166) of subjects, in dolichofacial types, 68.2% (n=45) presented a Type 2 canal, while in the mesofacial type, a lower prevalence of the bifid mandibular canal was observed (13.0%, n=23) than in the other facial types. The bifid canal showed significant association with facial type only (p<0.05), but no significant association was observed with the anterior loop type (p>0.05). Facial type is significantly associated with the path and morphological variations of the mandibular canal, independently of the side of the face studied, age, and sex.

Multimodality management of Spetzler-Martin Grade III arteriovenous malformations

2012 ◽  
Vol 116 (6) ◽  
pp. 1279-1288 ◽  
Author(s):  
Paritosh Pandey ◽  
Michael P. Marks ◽  
Ciara D. Harraher ◽  
Erick M. Westbroek ◽  
Steven D. Chang ◽  
...  
Keyword(s):  
Neurological Deficit ◽  
Arteriovenous Malformations ◽  
Venous Drainage ◽  
Neurological Deficits ◽  
Eloquent Cortex ◽  
Type 3 ◽  
Grade Iii

Object Grade III arteriovenous malformations (AVMs) are diverse because of their variations in size (S), location in eloquent cortex (E), and presence of central venous drainage (V). Because they may have implications for management and outcome, the authors evaluated these variations in the present study. Methods Between 1984 and 2010, 100 patients with Grade III AVMs were treated. The AVMs were categorized by Spetzler-Martin characteristics as follows: Type 1 = S1E1V1, Type 2 = S2E1V0, Type 3 = S2E0V1, and Type 4 = S3E0V0. The occurrence of a new neurological deficit, functional status (based on modified Rankin Scale [mRS] score) at discharge and follow-up, and radiological obliteration were correlated with demographic and morphological characteristics. Results One hundred patients (49 female and 51 male; age range 5–68 years, mean 35.8 years) were evaluated. The size of AVMs was less than 3 cm in 28 patients, 3–6 cm in 71, and greater than 6 cm in 1; 86 AVMs were located in eloquent cortex and 38 had central drainage. The AVMs were Type 1 in 28 cases, Type 2 in 60, Type 3 in 11, and Type 4 in 1. The authors performed embolization in 77 patients (175 procedures), surgery in 64 patients (74 surgeries), and radiosurgery in 49 patients (44 primary and 5 postoperative). The mortality rate following the management of these AVMs was 1%. Fourteen patients (14%) had new neurological deficits, with 5 (5%) being disabling (mRS score > 2) and 9 (9%) being nondisabling (mRS score ≤ 2) events. Patients with Type 1 AVMs (small size) had the best outcome, with 1 (3.6%) in 28 having a new neurological deficit, compared with 72 patients with larger AVMs, of whom 13 (18.1%) had a new neurological deficit (p < 0.002). Older age (> 40 years), malformation size > 3 cm, and nonhemorrhagic presentation predicted the occurrence of new deficits (p < 0.002). Sex, eloquent cortex, and venous drainage did not confer any benefit. In 89 cases follow-up was adequate for data to be included in the obliteration analysis. The AVM was obliterated in 78 patients (87.6%), 69 of them (88.5%) demonstrated on angiography and 9 on MRI /MR angiography. There was no difference between obliteration rates between different types of AVMs, size, eloquence, and drainage. Age, sex, and clinical presentation also did not predict obliteration. Conclusions Multimodality management of Grade III AVMs results in a high rate of obliteration, which was not influenced by size, venous drainage, or eloquent location. However, the development of new neurological deficits did correlate with size, whereas eloquence and venous drainage did not affect the neurological complication rate. The authors propose subclassifying the Grade III AVMs according to their size (< 3 and ≥ 3 cm) to account for treatment risk.

Proposal of Venous Drainage–Based Classification System for Carotid Cavernous Fistulae With Validity Assessment in a Multicenter Cohort

Neurosurgery ◽  
2015 ◽  
Vol 77 (3) ◽  
pp. 380-385 ◽  
Author(s):  
Ajith J. Thomas ◽  
Michelle Chua ◽  
Matthew Fusco ◽  
Christopher S. Ogilvy ◽  
R. Shane Tubbs ◽  
...  
Keyword(s):  
Endovascular Treatment ◽  
Classification System ◽  
Internal Carotid ◽  
Treatment Approach ◽  
Venous Drainage ◽  
Validity Assessment ◽  
Drainage Channels ◽  
Type 3

Abstract BACKGROUND: Carotid cavernous fistulae (CCFs) are most commonly classified based on arterial supply. Symptomatology and treatment approach, however, are largely influenced by venous drainage. OBJECTIVE: To propose an updated classification system using venous drainage. METHODS: CCFs with posterior/inferior drainage only, posterior/inferior and anterior drainage, anterior drainage only, and retrograde drainage into cortical veins with/without other drainage channels were designated as types 1, 2, 3, and 4, respectively. CCFs involving a direct connection between the internal carotid artery and cavernous sinus were designated as type 5. This system was retrospectively applied to 29 CCF patients. RESULTS: Our proposed classification was significantly associated with symptomatology (P.001). Type 2 was significantly associated with coexisting ocular/orbital and cavernous symptoms only (P.001), type 3 with ocular/orbital symptoms only (P.01), and type 4 demonstrated cortical symptoms with/without ocular/orbital and cavernous symptoms (P.01), respectively. There was a significant association of our classification system with the endovascular treatment approach (P.001). Types 1 and 2 were significantly associated with endovascular treatment through the inferior petrosal sinus (P.01). Type 3 was significantly associated with endovascular treatment through the ophthalmic vein (P.01) and type 5 with transarterial approach (P.01), respectively. Types 2 (27.6%) and 3 (34.5%) were most prevalent in this series, whereas type 1 was rare (6.9%), suggesting that some degree of thrombosis is present, with implications for spontaneous resolution. Type 2 CCFs demonstrated a trend toward partial resolution after endovascular treatment (P = .07). CONCLUSION: Our proposed classification system is easily applicable in clinical practice and demonstrates correlation with symptomatology, treatment approach, and outcome.

Abstract 1122‐000048: Endovascular Treatment for Spetzler‐Martin Grade III Pediatric Arteriovenous Malformations

2021 ◽  
Vol 1 (S1) ◽  
Author(s):  
Aaron Rodriguez Calienes ◽  
Aaron Rodriguez‐Calienes ◽  
Giancarlo Saal‐Zapata ◽  
Lilian Alvarez ◽  
Marco Malaga ◽  
...  
Keyword(s):  
Endovascular Treatment ◽  
Arteriovenous Malformations ◽  
Intraoperative Complications ◽  
Venous Drainage ◽  
Single Session ◽  
Complete Obliteration ◽  
Type 3 ◽  
Grade Iii

Introduction : Grade III Spetzler‐Martin (SM) brain arteriovenous malformations (AVMs) presents high variability in terms of size (S), angioarchitecture, flow characteristics, a frequent involvement of eloquent areas (E), and presence of central venous drainage (V). Therefore, this specific group fall into a gray zone in which the best treatment option is not stablished. Here, we aimed to assess the safety and efficacy of intent‐to‐cure embolization in pediatric grade III AVM management at two institutions. Methods : Pediatric patients (<18 years of age) with grade III AVMs treated with intent‐to‐cure embolization in two institutions between 2010 and 2019 were included. These two centers primarily perform endovascular treatment with intention to cure, which means that they attempt to occlude the maximal volume of nidus in a single session. Then, if the first session is not curative or the result is partial, a subsequent embolization is planned to completely occlude the AVM nidus. The clinical features, obliteration rates, and intraoperative complications were retrospectively collected from the clinical records. We categorized the AVMs based by the SM features: Type 1 = S1E1V1, Type 2 = S2E1V0, Type 3 = S2E0V1, and Type 4 = S3E0V0. The Institutional Review Boards approved this study. Results : Twenty‐seven children (19 females; mean age: 12 years, standard deviation: 3.9 years) with grade III AVMs underwent 47 embolization sessions. The most common presentation was intracranial hemorrhage (66%), and the majority (48%) were deep lesions (basal ganglia, corpus callosum, ventricle). The size of AVMs was <3 cm in 16 patients, 3 – 6 cm in 9, and >6 cm in 2; 21 AVMs were in eloquent cortex and 20 had deep venous drainage. The AVMs were Type 1 in 16 cases, Type 2 in 5, Type 3 in 4, and Type 4 in 2. Complete obliteration was achieved in 12 patients (44%), including 37% of AVMs exclusions after a single session. Eight (30%) patients had multiple embolizations. The AVM was obliterated after a single session in 10 patients (63%) with Type 1 AVMs (small lesions). The most common embolic agent employed was Squid (17/44), followed by Onyx (14/44) and Histoacryl (6/44). Intraoperative complications were reported in 5 (4 microperforations, 1 microcatheter rupture) out of 47 sessions (11%), with only one complication in the large AVM group (Types 2 ‐ 4). Deaths were not reported. Conclusions : Endovascular treatment with intent‐to‐cure of grade III SM AVMs in the pediatric population has demonstrated adequate complete obliteration rates with acceptable intraoperative complication rates. Therefore, long‐term follow‐up in this population is necessary in order to assess the real impact of embolization in terms of cure rates.

Anatomical variations of the external branch of the superior laryngeal nerve in relation to the inferior constrictor muscle: cadaveric dissection study

2012 ◽  
Vol 126 (9) ◽  
pp. 907-912 ◽  
Author(s):  
U Patnaik ◽  
A Nilakantan ◽  
T Shrivastava
Keyword(s):  
Superior Laryngeal Nerve ◽  
Anatomical Variations ◽  
Laryngeal Nerve ◽  
Rational Approach ◽  
Cadaveric Dissection ◽  
Constrictor Muscle ◽  
External Branch ◽  
Type 3

AbstractObjectives:To determine anatomical variations in the external branch of the superior laryngeal nerve in relation to the inferior constrictor muscle, and to propose a rational approach for the preservation of the nerve in thyroid surgery based on anatomical principles.Method:A cadaveric dissection study of the anatomy of the external branch of the superior laryngeal nerve in relation to the inferior constrictor muscle was conducted. Twenty-nine formalin-fixed cadavers of both sexes (age 50–70 years), with normal necks, were examined.Results:In relation to the Friedman classification, three anatomical variations of the external branch of the superior laryngeal nerve were found. Type 1 variation was found in 57.1 per cent of cases, type 2 in 26.8 per cent and type 3 in 16 per cent.Conclusion:The prevalence of type 3 variation of the external branch of the superior laryngeal nerve suggests that the nerve will not be encountered in a certain percentage of individuals as it lies under the cover of the inferior constrictor. Therefore, there is no justification for attempting to identify the nerve in all cases.

scholarly journals Anatomic Variations of the Superficial Middle Cerebral Vein: Embryologic Aspects of the Regressed Embryonic Tentorial Sinus

2005 ◽  
Vol 11 (2) ◽  
pp. 115-122 ◽  
Author(s):  
J. I. Chung ◽  
Y. C. Weon
Keyword(s):  
Cavernous Sinus ◽  
Venous Drainage ◽  
Middle Cranial Fossa ◽  
Transverse Sinus ◽  
Cerebral Vein ◽  
Anatomic Variations ◽  
Drainage Patterns ◽  
Developmental Regression ◽  
Superficial Middle Cerebral Vein ◽  
Tentorial Sinus

The embryonic tentorial sinus usually regressses during postnatal development, but its typical prenatal drainage patterns and intradural anastomoses can be depicted as various developmental phenotypic representations. Here, we tried to clarify the variant types of the superficial middle cerebral vein (SMCV) associated with the embryonic tentorial sinus. Total 41 patients and 82 hemispheres were included in this study. CT angiography was performed in all patients as screening for cerebrovascular disease or other intracranial disorders. A separate workstation and 3D software were used to evaluate the cranial venous systems with 3D volume rendering techniques, thin-slice MIP images, and MPR techniques for the analysis of its complicated angioarchitecture. Variations of the SMCV were classified according to the developmental alterations of the embryonic tentorial sinus, including sphenoparietal sinus (cranial remnant of tentorial sinus), basal sinus (floor of middle cranial fossa), petrosal and caudal remnant of the tentorial sinus. Secondary intradural anastomoses of cavernous and superior petrosal sinuses were also evaluated for the efferent pathways. The most frequent type of remnant tentorial sinus, sphenoparietal sinus was present in 49% (40/82) of hemispheres examined. Other regressed patterns of embryonic tentorial sinus were also identified in 38% (31/82): nine caudal remnant type around the transverse sinus, 12 petrosal type, one basal type, five unclassified cases, and mixed type were found in four cases. Secondary intradural cavernous sinus anastomosis was seen in 44% (36/82), however the most prevalent pattern was no anastomosis (46/82) with cavernous sinus. Only one case of superior petrosal sinus anastomosis was found in this series associated with basal sinus type. Anatomic variations of SMCV can be clearly demonstrated with embryologic aspects of the tentorial sinus according to its developmental regression and postnatal secondary adaptations of cerebral venous drainage.

FUNCTIONAL STATUS AND ADAPTIVE POTENTIAL IN FOREIGN STUDENTS WITH DIFFERENT TYPES OF VEGETATIVE REGULATION DURING TUITION

2020 ◽  
pp. 118-126
Author(s):  
A.M. Satarkulova
Keyword(s):  
Heart Rate ◽  
Foreign Students ◽  
Autonomic Regulation ◽  
The Body ◽  
Functional Changes ◽  
Different Types ◽  
Type 3 ◽  
Adaptive Abilities

The assessment and dynamic control over students’ status is a very important task. It allows timely detection of prenosological status prior to pathology and health maintenance in students. The objective of the paper is to assess the adaptive abilities of the body, to analyze changes in heart rate variability indicators in students with various types of autonomic regulation, to identify prenosological status and precursory pathological symptoms. Materials and Methods. The study enrolled 302 students from India, aged 21.54±1.43. Programming complex «Psychophysiologist» was used to register the main HRV parameters within 5 minutes. Health status was evaluated according to the index of functional changes and the scale of functional states. Results. N.I. Shlyk (2009) distinguished two groups of students with different types of autonomic regulation: type 1 (53 %) with moderate and type 2 (5 %) with marked characteristics of central regulation profile, type 3 (35 %) with moderate and type 4 (7 %) with marked characteristics of autonomous regulation profile. Main parameters of HRV and adaptation potential were defined for each student.All the parameters characterized functional and health status. Conclusions. It was shown that 82 % of trial subjects (type 1), 53 % (type 2), 94 % (type 3) and 95 % (type 4) demonstrated satisfactory adaptation and their physiological processes were at an optimal level. 18 % of students (type 1) demonstrated reduced adaptive abilities of the body. Moreover, they were under moderate stress. 47 % of subjects (type 2) were also under a significant stress, which was proven by excessively high SI, low SDNN and TP, and an increased index of functional changes. 5 % of students (type 4) revealed dysfunctional characteristics in the heart rhythm, peculiar to pathology. Keywords: foreign students, heart rate variability, types of autonomic regulation, adaptation potential, functional status. Оценка состояния студентов и динамический контроль за ним является важной задачей, поскольку позволяет своевременно выявлять у студентов донозологические состояния, предшествующие патологии, и способствовать сохранению здоровья. Цель. Оценка адаптивных возможностей организма, анализ изменений показателей вариабельности сердечного ритма у студентов с различными типами вегетативной регуляции, выявление донозологических состояний и ранних признаков патологии. Материалы и методы. В исследовании участвовало 302 студента в возрасте 21,54+1,43 года из Индии. Регистрировались основные параметры ВСР в течение 5 мин с использованием программно-аппаратного комплекса «Психофизиолог». Состояние и уровень здоровья оценивались по индексу функциональных изменений и шкале функциональных состояний. Результаты. По способу, предложенному Н.И. Шлык, выделены группы студентов с различными типами вегетативной регуляции: I (53 %) и II типы (5 %) – с умеренным и выраженным преобладанием центрального контура регуляции соответственно, III (35 %) и IV типы (7 %) – с умеренным и выраженным преобладанием автономного контура регуляции соответственно. У каждого из студентов определены основные параметры ВСР и адаптационного потенциала, характеризующие функциональное состояние и уровень здоровья. Выводы. Показано, что для 82 % обследуемых с I типом, 53 % со II типом, 94 % c III типом и 95 % с IV типом регуляции характерно состояние удовлетворительной адаптации, физиологические процессы сохраняются на оптимальном уровне. В группе студентов I типа у 18 % студентов адаптивные возможности организма снижены, выявлено состояние умеренного напряжения. У 47 % обследуемых II типа также зафиксировано состояние резко выраженного напряжения, индикатором которого является чрезмерно высокое значение SI, низкие величины SDNN и ТP, повышенное значение индекса функциональных изменений. В группе студентов с IV типом у 5 % учащихсяв регуляции ритма сердца выявлены дисфункциональные признаки, характерные для патологии. Ключевые слова: иностранные студенты, вариабельность сердечного ритма, типы вегетативной регуляции, адаптационный потенциал, функциональное состояние.

POLIOMYELITIS IN CANADIAN ESKIMOS: LABORATORY STUDIES. V. TYPE 1 AND TYPE 3 POLIOMYELITIS ANTIBODY LEVELS IN BAFFIN ISLAND ESKIMOS

1954 ◽  
Vol 32 (1) ◽  
pp. 119-125
Author(s):  
W. Wood ◽  
Eina M. Clark ◽  
F. T. Shimada ◽  
A. J. Rhodes
Keyword(s):  
Medical Records ◽  
Baffin Island ◽  
Tissue Cultures ◽  
Laboratory Studies ◽  
Poliomyelitis Virus ◽  
Antibody Titers ◽  
Antibody Levels ◽  
Type 3

Studies on the basic immunology of poliomyelitis in Canadian Eskimos have been continued. Some 87 sera collected from Eskimos at Pangnirtung, Baffin Island, have been examined for the presence of Type 1 and Type 3 poliomyelitis antibody by quantitative tests in tissue cultures. The same sera were previously examined for Type 2 antibody by quantitative tests in mice. The results of the three determinations are now presented together for comparison. These sera came from Eskimos aged 2 to 72 years of age. None of the Eskimos showed any evidence of paralysis. Examination of the medical records did not suggest that any paralytic disease had been present in this part of Baffin Island. Very few of the sera showed the presence of poliomyelitis antibody; thus, Type 1 antibody was demonstrated in the sera of 8%, Type 2 antibody in the sera of 9%, and Type 3 antibody in the sera of 14%. No significant number of Eskimos below the age of 45 years had acquired poliomyelitis antibody. The antibody titers mostly ranged between 10−1.0 and 10−2.0, and were significantly lower than the titers customarily found in recently paralyzed cases. These findings suggest that poliomyelitis infection occurred in Pangnirtung Eskimos many years before the date on which the samples were taken (1951). These results point to the worldwide prevalence of the three types of poliomyelitis virus.

Toward Personalized Treatment for Patients with Low von Willebrand Factor and Quantitative von Willebrand Disease

2021 ◽  
Vol 47 (02) ◽  
pp. 192-200
Author(s):  
James S. O'Donnell
Keyword(s):  
Von Willebrand Factor ◽  
Bleeding Risk ◽  
Von Willebrand Disease ◽  
Personalized Treatment ◽  
Treatment Plans ◽  
Type 3 ◽  
Von Willebrand ◽  
Willebrand Factor

AbstractThe biological mechanisms involved in the pathogenesis of type 2 and type 3 von Willebrand disease (VWD) have been studied extensively. In contrast, although accounting for the majority of VWD cases, the pathobiology underlying partial quantitative VWD has remained somewhat elusive. However, important insights have been attained following several recent cohort studies that have investigated mechanisms in patients with type 1 VWD and low von Willebrand factor (VWF), respectively. These studies have demonstrated that reduced plasma VWF levels may result from either (1) decreased VWF biosynthesis and/or secretion in endothelial cells and (2) pathological increased VWF clearance. In addition, it has become clear that some patients with only mild to moderate reductions in plasma VWF levels in the 30 to 50 IU/dL range may have significant bleeding phenotypes. Importantly in these low VWF patients, bleeding risk fails to correlate with plasma VWF levels and inheritance is typically independent of the VWF gene. Although plasma VWF levels may increase to > 50 IU/dL with progressive aging or pregnancy in these subjects, emerging data suggest that this apparent normalization in VWF levels does not necessarily equate to a complete correction in bleeding phenotype in patients with partial quantitative VWD. In this review, these recent advances in our understanding of quantitative VWD pathogenesis are discussed. Furthermore, the translational implications of these emerging findings are considered, particularly with respect to designing personalized treatment plans for VWD patients undergoing elective procedures.

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