Interdisciplinary innovative approach to an aggressive ossifying fibroma case: Integrating 3D surgery and customized reconstruction in maxillofacial and dental care.

Aggressive ossifying fibroma is a benign fibro-osseous disorder characterized by its aggressive behavior, which complicates its management. In this article, we present a case involving the recurrence of this condition in the maxillary region, with orbital and dental involvement, in a patient who had previously undergone surgery and reconstruction with a microvascularized free fibula flap. A multidisciplinary approach involving maxillofacial surgery and dentistry was employed to deliver a customized and entirely satisfactory solution for the patient. The use of 3D surgery was integral to our approach, encompassing pre-surgical digital planning and the transfer of this planning to the operating room via navigation software. Customized surgical cutting guides facilitated precise resection, while a personalized polyether ether ketone (PEEK) prosthesis was utilized for reconstruction of the malar and infraorbital region. Pre-prosthetic computer-aided design/computer-aided manufacturing (CAD/CAM) surgery, along with dental rehabilitation using transepithelial abutments and dental prostheses on a titanium framework, were employed for dental restoration. During the postoperative period, mobility in the reconstructed maxilla was observed due to the loss of support from the initial reconstruction plate. This was addressed by replacing the plate with a custom-made titanium plate, designed to accommodate the location of the transepithelial abutments and prevent disruption of the dental rehabilitation. This case demonstrates the potential of new technologies when applied within the collaborative framework of maxillofacial surgeons and dentists, enabling effective and definitive solutions in complex reconstruction cases. Key words:Aggressive ossifying fibroma; 3D surgery; customized reconstruction; complex dental reconstruction.

Consensus Report and Recommendations on the Management of Late-stage Internal Derangement of the Temporomandibular Joint.

Introduction: This report investigates late-stage internal derangement (ID) of the temporomandibular joint (TMJ) with the aim of establishing a more effective and personalized treatment protocol to improve patients' quality of life (QoL). Material and methods: A consensus was reached among maxillofacial surgeons specializing in LSID, based on a literature research and collective expert experience following the Delphi method. Consensus was considered to be achieved when a response received at least 80% of votes. Results: Four expert groups were established, respectively, focusing on diagnosis, minimally invasive surgery (MIS), open surgery and joint replacement. A comprehensive approach to late-stage ID of the TMJ requires a consensus report. This underscores the need for a personalized treatment plan, considering the variability in clinical presentations and progression of this pathology. Our recommendations aim to optimize clinical outcomes and enhance patient QoL.

Anesthetic Management of a Patient with Madelung's Disease Presenting with a Giant Cervical Lipoma: A Case Report.

Madelung's disease is characterized by the presence of multiple symmetric and nonencapsulated facial, neck, and upper trunk lipomas. Depending on the location and size of lipomas, there may be concerns for difficult airway management and cardiovascular collapse. We present the anesthetic management case of a patient with cervical and mediastinal lipomas who underwent elective cervical lipoma resection.

Surface scanned 3D designed customized chinstrap for the treatment of intraoral dehiscence in polytraumatized patient. Technical note.

The advent of 3D surgical technology has revolutionized personalized medicine, enabling the development of tailored solutions for individual patients. This technical note presents the application of 3D technology in designing a customized chin guard using flexible 3D resin. The process involves surface scanning the lower facial region of a polytraumatized patient with a structured-light surface 3D scanner, generating a detailed point cloud. The acquired data undergoes meticulous processing within an specific professional software, including erasing unwanted portions, aligning frames, and mesh consolidation. Subsequently, the mesh is exported as an STL file and further refined using a 3D mesh management software. A customized chin support is designed for the specific patient's needs, exported in STL format, and 3D printed using a stereolithography (SLA) printer with Flexible 80A resin. Post-printing procedures involve washing and curing to ensure biocompatibility and optimal mechanical characteristics. The resultant customized chin guard, attached to elastic support straps, offers a precise fit to the patient's anatomy, enhancing comfort and allowing for extended wear. This innovative approach addresses the challenge of surgical intraoral wound dehiscence in a polytraumatized patient, showcasing the potential of 3D technology in personalized medical solutions for complex cases. Key words:Surface scanner, 3D surgery, customized surgery, chinstrap.

Virtual Surgical Planning for Temporomandibular Joint Reconstruction with Stock TMJ Prostheses: Pilot Study.

The temporomandibular joint (TMJ) is one of the most complex joints in the human anatomy. In advanced degenerative stages, conservative or minimally invasive surgical therapies have failed to restore joint function, and joint replacement with prostheses has been required. Stock prostheses, compared to custom-made prostheses, are much less expensive and require less pre-operative preparation time. Four patients followed for years for temporomandibular dysfunction and previously operated on by arthroscopy or open joint surgery that have been reconstructed with stock TMJ prostheses (STMJP) through virtual surgical planning (VSP) and an STL model with surgical and positioning guides were included. The median follow-up was 15 months; the median number of previous TMJ surgeries was 2. The mean preoperative MIO was 24.6 mm and at longest follow-up was 36.4 mm. The median preoperative TMJ pain score was 8, and the median postoperative TMJ pain was 3. All patients have improved their mandibular function with a clear improvement of their initial situation. In conclusion, we believe that stock TMJ prostheses with virtual surgical planning and surgical guides are a good alternative for TMJ reconstruction at the present time. Nonetheless, prospective and randomized trials are required with long-term follow up to assess their performance and safety.

3D designed in-house positioning and cutting guide system for genioplasty surgery. Technical note and case report.

Advances in 3D printing technology have the potential to significantly improve the workflow of maxillofacial surgical planning. In-house fabricated custom positioning and cutting guides allow for intraoperative reproduction of pre-planned osteotomy cuts, which can result in greater surgical accuracy and patient safety while maintaining an acceptable cost-effectiveness ratio. The design and creation of the customized surgical guides is performed in our hospital fab lab, which allows time savings, from an average of 10 days to just 24 hours, and a cost reduction of more than 90%. The process begins with the import of the pre-surgical facial CT scan into 3D software that allows to perform the surgical cuts virtually and the manipulation of the segments. Once the virtual planning of the surgery has been performed, the next step is the creation of the cutting and positioning guides. The final step is the printing of the guides in surgical resin and their sterilization. In addition, post-surgical models can be 3D printed to pre-mold the plates on them, which saves surgical time. The mentoplasty surgery is a simple example of how 3D surgery can be applied to maxillofacial surgery in an efficient way obtaining all the advantages of customized surgery with a limited investment in time and resources. Key words:3Dsurgery, customized, personalized medicine, genioplasty, surgical guides, in house.

Depth of Invasion: Influence of the Latest TNM Classification on the Prognosis of Clinical Early Stages of Oral Tongue Squamous Cell Carcinoma and Its Association with Other Histological Risk Factors.

BACKGROUND: The American Joint Committee on Cancer (AJCC), in its 8th edition, introduces modifications to the previous TNM classification, incorporating tumour depth of invasion (DOI). The aim of this research is to analyse the prognosis (in terms of disease-free survival and overall survival) of clinical early stage (I and II) squamous cell carcinomas of the oral tongue according to the DOI levels established by the AJCC in its latest TNM classification to assess changes to the T category and global staging system and to evaluate the association between DOI and other histological risk factors. METHODS: A retrospective longitudinal observational study of a series of cases was designed. All patients were treated with upfront surgery at our institution between 2010 and 2019. The variables of interest were defined and classified into four groups: demographic, clinical, histological and evolutive control. Univariate and multivariate analyses were carried out and survival functions were calculated using the Kaplan-Meier method. Statistical significance was established for p values below 0.05. RESULTS: Sixty-one patients were included. The average follow-up time was 47.42 months. Fifteen patients presented a loco-regional relapse (24.59%) and five developed distant disease (8.19%). Twelve patients died (19.67%). Statistically significant differences were observed, with respect to disease-free survival (p = 0.043), but not with respect to overall survival (p = 0.139). A total of 49.1% of the sample upstaged their T category and 29.5% underwent modifications of their global stage. The analysis of the relationship between DOI with other histological variables showed a significant association with the presence of pathological cervical nodes (p = 0.012), perineural invasion (p = 0.004) and tumour differentiation grade (p = 0.034). Multivariate analysis showed association between depth of invasion and perineural invasion. CONCLUSIONS: Depth of invasion is a histological risk factor in early clinical stages of oral tongue squamous cell carcinoma. Depth of invasion impacts negatively on patient prognosis, is capable per se of modifying the T category and the global tumour staging, and is associated with the presence of cervical metastatic disease, perineural invasion and tumoural differentiation grade.

HPV infection in oral cancer, our experience: prevalence, clinical implications, and current vaccination program in Spain.

Background: Oral cancer is the 11th most common type of cancer in the world, with established major risk factors as tobacco and alcohol, and recently included high-risk human papillomavirus types 16 and 18. HPV types 16 and 18 are the etiologic agents of cervical cancers and a proportion of oropharyngeal cancers. However, the picture of HPV and the clinical implications of oral cancers are not clear with most reports combining oral cancer data with head and neck cancers. It has been confirmed as a favorable prognostic factor in oropharyngeal cancer. However, the prognostic value of HPV in oral squamous cell carcinoma is still unclear. Material and Methods: The main objective of this article is to present the evidence encountered following a bibliographical review of recent publications specifically related to oral cancer and its differences from oropharyngeal cancer. The secondary goals are to present the findings of a five-year retrospective observational study of the prevalence of HPV infection in oral cancer patients treated by the Oral and Maxillofacial Surgery Department at La Paz University Hospital (Madrid, Spain), and finally, we to evaluate and compare our country's HPV prevention program in comparison to other European countries. Results: According to the review of the literature, HPV positive oral squamous cell carcinoma is associated with significantly decreased overall survival and distant control. Bibliographic review suggest HPV infection can be used as a negative prognostic factor in oral squamous cell carcinoma. Conclusions: As regards diagnostic testing for HPV, it should be extended to as many cases of oral cavity squamous cell carcinoma as possible, especially in those with risk factors. The current vaccination program in Spain does not have adequate coverage and is significantly under the level of other European Union countries; it should be expanded and catch-up strategies should be included. Key words:HPV, OSSC, Papillomavirus, oral carcinoma, prevention.

Use of surgical bone cement to increase the projection of the nasomaxillary buttress in a case of orthognathic surgery. Technical note.

The soft tissue outcome of the projection at the level of the nasomaxillary buttress is difficult to manage in cases of severe hypoprojection, being orthognathic surgery resolutive at the occlusal level but sometimes insufficient at the level of esthetic outcome. The literature describes the use of alloplastic prostheses and autologous bone grafts, but there are few documented cases of the use of premolded surgical cement for this purpose. The main advantage of the use of bone cement over the alternatives described is its ability to be premolded for customization, low cost, easy availability, speed of preparation and minimal comorbidity. This technical note describes the surgical steps and outcome of the use of surgical bone cement for projection augmentation at this level, including notes on preparation, premolding and fixation. Key words:Orthognathic surgery, maxillary surgery, surgical bone cement, nasomaxillary buttress.

A Quantitative and Qualitative Clinical Validation of Soft Tissue Simulation for Orthognathic Surgery Planning.

The purpose of this study was to perform a quantitative and qualitative validation of a soft tissue simulation pipeline for orthognathic surgery planning, necessary for clinical use. Simulation results were retrospectively obtained in 10 patients who underwent orthognathic surgery. Quantitatively, error was measured at 9 anatomical landmarks for each patient and different types of comparative analysis were performed considering two mesh resolutions, clinically accepted error, simulation time and error measured by means of percentage of the whole surface. Qualitatively, evaluation and binary questions were asked to two surgeons, both before and after seeing the actual surgical outcome, and their answers were compared. Finally, the quantitative and qualitative results were compared to check if these two types of validation are correlated. The quantitative results were accurate, with greater errors corresponding to gonions and lower lip. Qualitatively, surgeons answered similarly mostly and their evaluations improved when seeing the actual outcome of the surgery. The quantitative validation was not correlated to the qualitative validation. In this study, quantitative and qualitative validations were performed and compared, and the need to carry out both types of analysis in validation studies of soft tissue simulation software for orthognathic surgery planning was proved.

Virtual Surgical Planning and Customized Subperiosteal Titanium Maxillary Implant (CSTMI) for Three Dimensional Reconstruction and Dental Implants of Maxillary Defects after Oncological Resection: Case Series.

Maxillectomies cause malocclusion, masticatory disorders, swallowing disorders and poor nasolabial projection, with consequent esthetic and functional sequelae. Reconstruction can be achieved with conventional approaches, such as closure of the maxillary defect by microvascular free flap surgery or prosthetic obturation. Four patients with segmental maxillary defects that had been reconstructed with customized subperiosteal titanium maxillary implants (CSTMI) through virtual surgical planning (VSP), STL models and CAD/CAM titanium mesh were included. The smallest maxillary defect was 4.1 cm and the largest defect was 9.6 cm, with an average of 7.1 cm. The reconstructed maxillary vertical dimension ranged from 9.3 mm to 17.4 mm, with a mean of 13.17 mm. The transverse dimension of the maxilla at the crestal level was attempted to be reconstructed based on the pre-excision CT scan, and these measurements ranged from 6.5 mm in the premaxilla area to 14.6 mm at the posterior level. All patients were rehabilitated with a fixed prosthesis on subperiosteal implants with good esthetic and functional results. In conclusion, we believe that customized subperiosteal titanium maxillary implants (CSTMI) are a safe alternative for maxillary defects reconstruction, allowing for simultaneous dental rehabilitation while restoring midface projection. Nonetheless, prospective and randomized trials are required with long-term follow-up, to assess its long-term performance and safety.

ACOG and local diagnostic criteria for hypertensive disorders of pregnancy (HDP) in La Paz-El Alto, Bolivia: A retrospective case-control study.

Background: Hypertensive disorders of pregnancy (HDP) are a leading cause of maternal death in low- to middle-income countries (LMIC). The American College of Obstetricians and Gynecologists (ACOG) updated diagnostic guidelines to align signs and symptoms with those associated with maternal death. We performed an observational study to ask whether ACOG guidelines were employed and associated with adverse outcomes in La Paz-El Alto, Bolivia, an LMIC. Methods: Medical records for all HDP discharge diagnoses (n = 734) and twice as many controls (n = 1647) were reviewed for one year at the three largest delivery sites. For the 690 cases and 1548 controls meeting inclusion criteria (singleton, 18-45 maternal age, local residence), health history, blood pressures, symptoms, lab tests, HDP diagnoses (i.e., gestational hypertension [GH]; preeclampsia [PE]; haemolysis, low platelets, high liver enzymes [HELLP] syndrome, eclampsia), and adverse outcomes were recorded. Bolivian diagnoses were compared to ACOG guidelines using accuracy analysis and associated with adverse outcomes by logistic regression. Findings: Both systems agreed with respect to eclampsia, but only 27% of all Bolivian HDP diagnoses met ACOG criteria. HDP increased adverse maternal- or perinatal-outcome risks for both systems, but ACOG guidelines enabled more pre-delivery diagnoses, graded maternal-risk assessment, and targeting of HDP terminating in maternal death. Interpretation: Bolivia diagnoses agreed with ACOG guidelines concerning end-stage disease (eclampsia) but not the other HDP due mainly to ACOG's recognition of a broader range of severe features. ACOG guidelines can aid in identifying pregnancies at greatest risk in LMICs, where most maternal and perinatal deaths occur. Funding: NIH TW010797, HD088590, HL138181, UL1 TR002535.

Transplantation of hematopoietic progenitors in myelofibrosis.

The objective of this work is to generate recommendations on the management of allogeneic stem cell transplantation (allo-SCT) in primary myelofibrosis (PMF). A comprehensive systematic review of articles published between 1999 and 2015 (January) was used as a source of scientific evidence. The recommendations were produced through a Delphi process involving a panel of 23 experts appointed by the European LeukemiaNet and the European Blood and Marrow Transplantation Group. Key questions included patient selection, donor selection, pre-transplant management, conditioning regimen, post-transplant management, prevention, and management of post-transplant relapse. Patients with intermediate-2 or high-risk disease and age < 70 years should be considered candidates for allo-SCT. Patients with intermediate-risk 1 disease and age < 65 years should be considered candidates if they have refractory transfusion-dependent anemia, or a peripheral blood (PB) blast percentage > 2%, or adverse cytogenetics. Splenectomy before transplantation must be decided on a case-by-case basis. Patients with intermediate-2 or high-risk disease who lack a human leukocyte antigen (HLA)-matched sibling or unrelated donor should be enrolled in a protocol that uses HLA non-identical donors. PB was considered the most appropriate source of hematopoietic stem cells for transplants from HLA-matched unrelated donors and siblings. The optimal intensity of the conditioning regimen has yet to be defined. Strategies such as discontinuation of immunosuppressive drugs, infusion of donor lymphocytes, or both were considered adequate to prevent clinical relapse. In conclusion, we provide consensus-based recommendations aimed at optimizing allo-SCT in PMF. Unmet clinical needs were highlighted.

El objetivo de este trabajo es generar recomendaciones sobre el manejo del trasplante alogénico de células madre (alo-SCT) en la mielofibrosis primaria (MFP). Se utilizó una revisión sistemática integral de artículos publicados entre 1999 y 2015 (enero) como fuente de evidencia científica. Las recomendaciones se produjeron mediante un proceso Delphi en el que participó un panel de 23 expertos designados por la European LeukemiaNet y el European Blood and Marrow Transplantation Group. Las preguntas clave incluyeron la selección de pacientes, la selección de donantes, el manejo previo al trasplante, el régimen de acondicionamiento, el manejo posterior al trasplante, la prevención y el manejo de la recaída después del trasplante. Los pacientes con enfermedad de riesgo intermedio 2 o alto y edad < 70 años deben ser considerados candidatos para alo-SCT. Los pacientes con enfermedad de riesgo intermedio 1 y edad < 65 años deben ser considerados candidatos si presentan anemia refractaria dependiente de transfusiones, o un porcentaje de blastos en sangre periférica > 2%, o citogenética adversa. La esplenectomía previa al trasplante debe decidirse caso por caso. Los pacientes con enfermedad de riesgo intermedio 2 o alto que carecen de un hermano compatible con el antígeno leucocitario humano (HLA) o de un donante no emparentado deben inscribirse en un protocolo que utilice donantes no idénticos de HLA. PB se consideró la fuente más apropiada de células madre hematopoyéticas para trasplantes de hermanos y donantes no emparentados compatibles con HLA. La intensidad óptima del régimen de acondicionamiento aún debe definirse. Se consideraron adecuadas estrategias como la suspensión de los fármacos inmunosupresores, la infusión de linfocitos del donante o ambas para evitar la recaída clínica. En conclusión, proporcionamos recomendaciones basadas en consenso destinadas a optimizar el alo-SCT en MFP. Se destacaron las necesidades clínicas insatisfechas.

Essential thrombocythaemia.

Essential thrombocythemia (ET) is a chronic Philadelphia-negative myeloproliferative neoplasm that has its main involvement in the megakaryopoietic lineage, generating sustained thrombocytosis in peripheral blood and an increase in the number of mature megakaryocytes in the bone marrow. In addition to marked thrombocytosis, it is characterized by increased thrombotic or hemorrhagic risk and the presence of constitutional symptoms. Patients with ET have a low but known risk of disease progression to myelofibrosis and/or acute leukemia. The diagnosis is made based on the 2016 WHO criteria. At present, available treatments for patients with ET are mainly aimed at minimizing the risk of thrombosis and/or bleeding.

La trombocitemia esencial (TE) es una neoplasia mieloproliferativa crónica Filadelfia negativa que tiene su principal involucro en la línea megacariopoyética, generando trombocitosis sostenida en la sangre periférica y un incremento en el número de megacariocitos maduros en médula ósea. Además de una marcada trombocitosis, se caracteriza por un mayor riesgo trombótico o hemorrágico y la presencia de síntomas constitucionales. Los pacientes con TE tienen un riesgo bajo, pero conocido, de evolución de la enfermedad a mielofibrosis y/o leucemia aguda. El diagnóstico se realiza con base en los criterios de la Organización Mundial de la Salud del 2016. Los tratamientos actualmente disponibles para los pacientes con TE están dirigidos principalmente a minimizar el riesgo de trombosis y/o hemorragia.

Pregnancy in myeloproliferative neoplasms.

Myeloproliferative neoplasms (MPN) are associated with a significant risk of thrombosis and the hypercoagulable environment of pregnancy increases this risk. The most frequent gestational complications consist of spontaneous abortion, thrombosis, bleeding, and hypertensive disease of pregnancy. Treatment depends on thrombotic risk, gestational trimester, and myeloproliferative neoplasm.

Las neoplasias mieloproliferativas (NMP) están asociadas a un riesgo notable de trombosis y el entorno de hipercoagulabilidad propio del embarazo aumenta este riesgo. Las complicaciones gestacionales más frecuentes consisten en: aborto espontáneo, trombosis, sangrado y enfermedad hipertensiva del embarazo. El tratamiento depende del riesgo trombótico, trimestre gestacional y neoplasia mieloproliferativa.

Polycythemia vera.

Polycythemia vera (PV) is mainly characterized by erythrocytosis, thrombotic and hemorrhagic predisposition, a variety of symptoms, and cumulative risks of fibrotic progression and/or leukemic evolution over time. The diagnosis is made based on the 2016 WHO criteria. The treatment of PV focuses on rapidly reducing the erythrocyte mass, either by means of phlebotomies or with cytoreductive treatment, and the reduction of thrombotic risk by correcting cardiovascular risk factors and the use of platelet antiaggregants.

La policitemia vera (PV) se caracteriza principalmente por eritrocitosis, predisposición trombótica y hemorrágica, una variedad de síntomas y riesgos acumulativos de progresión fibrótica y/o evolución leucémica a lo largo del tiempo. El diagnóstico se realiza con base en los criterios de la Organización Mundial de la Salud del 2016. El tratamiento de la PV se centra en reducir rápidamente la masa eritrocitaria, ya sea por medio de flebotomías o con tratamiento citorreductor, y la disminución del riesgo trombótico mediante la corrección de factores de riesgo cardiovascular y el uso de antiagregantes plaquetarios.

Symptom control.

In addition to symptoms secondary to splenomegaly, microvascular abnormalities, and thrombohemorrhagic complications, patients with MPN may experience a significant symptom burden attributed to an increase in circulating inflammatory cytokines. These symptoms can be severe and limit quality of life. Therefore, in addition to the prevention of complications, one of the objectives of the treatment of MPN is the control of symptoms.

Además de la sintomatología secundaria a la esplenomegalia, a las alteraciones microvasculares y a las complicaciones trombohemorrágicas, los pacientes con neoplasias mieloproliferativas (NMP) pueden experimentar una importante carga sintomática atribuida a un aumento de citocinas inflamatorias circulantes. Estos síntomas pueden ser severos y limitar la calidad de vida. Por ello, además de la prevención de las complicaciones, uno de los objetivos del tratamiento de las NMP es el control de los síntomas.

Thrombotic events.

Major thrombotic complications in myeloproliferative neoplasms (MPNs) represent an important clinical problem due to their high morbidity, the complexity of their management, and their associated mortality. The appearance of a thrombosis implies a high thrombotic risk stratification of the MPN and determines the initiation or optimization of cytoreductive treatment and the use of antiplatelet or anticoagulant therapy as secondary prophylaxis. The incidence of thrombosis at the time of diagnosis is higher than during the course of the disease, being located in the arterial territory in 60-70% of cases. Once thrombosis has occurred, up to 20-33% of patients experience thrombotic recurrence in the same initial vascular territory.

Las complicaciones trombóticas mayores en las neoplasias mieloproliferativas (NMP) representan un importante problema clínico debido a su elevada morbilidad, la complejidad de su manejo y su mortalidad asociada. La aparición de una trombosis comporta una estratificación de alto riesgo trombótico de la NMP y determina el inicio o la optimización del tratamiento citorreductor y el uso de terapia antiplaquetaria o anticoagulante como profilaxis secundaria. La incidencia de trombosis en el momento del diagnóstico es mayor que durante la evolución de la enfermedad, localizándose en territorio arterial en el 60-70% casos. Una vez se ha producido una trombosis, hasta el 20-33% de los pacientes sufre una recurrencia trombótica en el mismo territorio vascular inicial.

Myelofibrosis: diagnosis and treatment.

Myelofibrosis (MF) is a BCR-ABL1-negative myeloproliferative neoplasm characterized by clonal myeloproliferation, dysregulated kinase signaling, and release of abnormal cytokines. In recent years, important progress has been made in the knowledge of the molecular biology and the prognostic assessment of MF. Conventional treatment has limited impact on the patients' survival; it includes a wait-and-see approach for asymptomatic patients, erythropoiesis-stimulating agents, androgens, or immunomodulatory agents for anemia, cytoreductive drugs such as hydroxyurea for the splenomegaly and constitutional symptoms, and splenectomy or radiotherapy in selected patients. The discovery of the Janus kinase (JAK) 2 mutation triggered the development of molecular targeted therapy of MF. The JAK inhibitors are effective in both JAK2-positive and JAK2-negative MF; one of them, ruxolitinib, is the current best available therapy for MF splenomegaly and constitutional symptoms. Although ruxolitinib has changed the therapeutic scenario of MF, there is no clear indication of a disease-modifying effect. Allogeneic stem cell transplantation remains the only curative therapy of MF, but due to its associated morbidity and mortality, it is usually restricted to eligible high- and intermediate-2-risk MF patients. To improve current therapeutic results, the combination of JAK inhibitors with other agents is currently being tested, and newer drugs are being investigated.

La mielofibrosis (MF) es una neoplasia mieloproliferativa negativa para BCR-ABL1 caracterizada por mieloproliferación clonal, señalización de cinasa desregulada y liberación de citocinas anormales. En los últimos años se han realizado importantes avances en el conocimiento de la biología molecular y la valoración pronóstica de la MF. El tratamiento convencional tiene un impacto limitado en la supervivencia de los pacientes; incluye un enfoque de espera para pacientes asintomáticos, agentes estimulantes de la eritropoyesis, andrógenos o agentes inmunomoduladores para la anemia, fármacos citorreductores como la hidroxiurea para la esplenomegalia y los síntomas constitucionales, y esplenectomía o radioterapia en pacientes seleccionados. El descubrimiento de la mutación Janus cinasa (JAK) 2 desencadenó el desarrollo de la terapia dirigida molecular de la MF. Los inhibidores de JAK son efectivos tanto en MF con JAK2 positivo como con JAK2 negativo; uno de ellos, el ruxolitinib, es la mejor terapia disponible actualmente para la esplenomegalia y los síntomas constitucionales de la MF. Sin embargo, aunque el ruxolitinib ha cambiado el escenario terapéutico de la MF, no hay indicios claros de un efecto modificador de la enfermedad. El alotrasplante de células madre sigue siendo la única terapia curativa de la MF, pero debido a su morbilidad y mortalidad asociadas, generalmente se restringe a pacientes elegibles con MF de riesgo alto e intermedio 2. Para mejorar los resultados terapéuticos actuales, actualmente se está probando la combinación de inhibidores de JAK con otros agentes y se están investigando fármacos más nuevos.

Intraosseous Venous Malformation of the Zygomatic Bone: Comparison between Virtual Surgical Planning and Standard Surgery with Review of the Literature.

Intraosseous venous malformations affecting the zygomatic bone are infrequent. Primary reconstruction is usually accomplished with calvarial grafts, although the use of virtual surgical planning, cutting guides and patient-specific implants (PSI) have had a major development in recent years. A retrospective study was designed and implemented in patients diagnosed with intraosseous venous malformation during 2006-2021, and a review of the scientific literature was also performed to clarify diagnostic terms. Eight patients were treated, differentiating two groups according to the technique: four patients were treated through standard surgery with resection and primary reconstruction of the defect with calvarial graft, and four patients underwent resection and primary reconstruction through virtual surgical planning (VSP), cutting guides, STL models developed with CAD-CAM technology and PSI (titanium or Polyether-ether-ketone). In the group treated with standard surgery, 75% of the patients developed sequelae or morbidity associated with this technique. The operation time ranged from 175 min to 210 min (average 188.7 min), the length of hospital ranged from 4 days to 6 days (average 4.75 days) and the postoperative CT scan showed a defect surface coverage of 79.75%. The aesthetic results were "excellent" in 25% of the patients, "good" in 50% and "poor" in 25%. In the VSP group, 25% presented sequelae associated with surgical treatment. The operation time ranged from 99 min to 143 min (average 121 min), the length of hospital stay ranged from 1 to 2 days (average of 1.75 days) and 75% of the patients reported "excellent" results. Postoperative CT scan showed 100% coverage of the defect surface in the VSP group. The multi-stage implementation of virtual surgical planning with cutting guides, STL models and patient-specific implants increases the reconstructive accuracy in the treatment of patients diagnosed with intraosseous venous malformation of the zygomatic bone, reducing sequelae, operation time and average hospital stay, providing a better cover of the defect, and improving the precision of the reconstruction and the aesthetic results compared to standard technique.