Morphological description of the temporomandibular joint ligaments in domestic pigs.

The temporomandibular joint (TMJ) ligaments play crucial roles in its function or dysfunction. The objective of this study was to describe the macro and microscopic morphology of these ligaments in domestic pigs, aiming to: (1) expand knowledge about the species; (2) provide anatomical references for advancing veterinary therapy and utilizing pigs as animal models in craniofacial research. Heads of young Sus scrofa domesticus were dissected to identify TMJ ligaments. Fragments of these ligaments were collected and processed for subsequent histological analysis with Haematoxylin and eosin staining. The results were qualitatively described. Pigs exhibited a TMJ reinforced by three individualized capsular ligaments: a lateral ligament, attaching to the ventral margin of the zygomatic process of the temporal bone and the lateral margin of the mandibular neck; a caudomedial ligament, attaching to the retroarticular process of the squamous part of the temporal bone and the caudomedial margin of the mandibular neck and a caudolateral ligament, attaching to the ventral margin of the base of the zygomatic process of the temporal bone and the caudal margin of the mandibular neck. The lateral ligament exhibited a greater constitution of dense irregular connective tissue, while the caudomedial and caudolateral ligaments showed a greater constitution of dense regular connective tissue. It is concluded that the TMJ of pigs presents one more ligament than horses, cattle, dogs, cats and what has been described for pigs themselves. We believe these results may contribute to the improvement of veterinary clinical and surgical therapy overall, as well as provide essential morphological information for a better interpretation and application of interspecies results in craniofacial research using pigs as an experimental model, as in the case of humans.

Impact of COVID-19 Pandemic on Cleft Lip/Palate Surgery in Brazil: Assessing the Current Landscape.

OBJECTIVE: This study aims to evaluate the impact of COVID-19 and the current situation for cleft lip/palate treatment surgeries within Brazil's public health system. DESIGN: Our retrospective study analyzed CL/P corrective surgeries in Brazil's health system using DATASUS TabNet data from March 2020 to December 2022, with historical data from January 2016 to February 2020. We employed ARIMA analysis to estimate pandemic-related surgery cancellations. RESULTS: In 2020, 1992 (CI 95%: 989-2995) CL/P surgeries were not conducted due to pandemics, a 44.1% (CI 95%: 28.1-54.2%) decrease compared to expectations for march to December 2020. Between the onset of the pandemic in Brazil and the end of 2022, 10,643 surgeries were performed in the country, representing a 33.8% shortfall compared to the expected number for the period (16,076; 95% CI: 9697-22,456). CONCLUSION: The study highlights COVID-19's impact on CL/P surgeries in Brazil. Post-pandemic, surgeries increased but regional disparities remain, urging collaborative efforts to improve services and support affected patients.

Complex Craniosynostosis in Pitt-Hopkins Syndrome: Case Report in Twins.

INTRODUCTION: Pitt-Hopkins syndrome (PTHS) is a rare genetic syndrome associated with neurodevelopmental disorders and craniofacial dysmorphisms caused by variations in the TCF4 transition factor. The aim of this article was to report the case of two twin infants diagnosed with PTHS, confirmed by the identification of a heterozygous pathogenic variant in the TCF4 gene through DNA extracted from a buccal swab. CASE PRESENTATION: Both infants presented with craniofacial asymmetry with a metopic crest and cranial deformity. During the diagnostic investigation, computed tomography with three-dimensional reconstruction of the skull showed premature fusion of the left coronal and metopic sutures in both twins. They underwent craniofacial reconstruction at the 9th month of age using a combination of techniques. The postoperative outcomes were satisfactory in both cases. CONCLUSION: To the best of our knowledge, this is the first case report to describe the occurrence of complex craniosynostosis (CCS) in children with PTHS. Further studies are needed to determine whether the co-occurrence of PTHS and CCS described here indicates an association or is explained by chance.

Growing skull fracture in a child with Ehlers-Danlos syndrome: case report and literature review.

INTRODUCTION: Growing skull fracture (GSF) is a rare complication of head trauma in the pediatric population, commonly observed in children younger than 3 years. DISCUSSION: In this report, the authors describe a case of a 3-year-old male child, with clinical features of Ehlers-Danlos syndrome (EDS), who developed a GSF in frontal bone after a crib fall, treated with duraplasty and cranioplasty with autologous craft. Here, pertinent literature was reviewed with an emphasis on surgical techniques, and correlation with the mentioned syndrome. CONCLUSION: This is the first case of GSF in association with EDS in the literature. The relevance of the case described concerns the rarity of the condition itself, the atypical presentation, and the intraoperative findings, which showed the important fragility of the dura mater, probably due to EDS. Therefore, this syndrome, besides having influenced the pathogenesis, was also a challenging factor in the surgical treatment.

Optic nerve sheath diameter with intracranial pressure monitoring: a non-invasive method to follow children with craniosynostosis.

Craniosynostosis (CS) is associated with increased intracranial pressure (ICP) and this elevation is of vital importance in children. Bedside optic nerve sheath diameter (ONSD) on ocular ultrasonography has begun to be increasingly used in recently. A patient who was diagnosed with CS, standard anesthesia monitoring and anesthesia were performed. Before and after the surgery, ONSD measurement was performed to follow the changes in ICP. ONSD measurement can be used as an effective, non-invasive, repeated, and easy-to-apply method to monitor the changes in the ICP in pediatric patients with CS who are planned to undergo craniectomy.

La craneosinostosis (SC) se asocia con un aumento de la presión intracraneal (PIC) y esta elevación es de vital importancia en los niños. El diámetro de la vaina del nervio óptico al lado de la cama (ONSD) en la ecografía ocular ha comenzado a usarse cada vez más recientemente. A un paciente al que se le diagnosticó SC, se le realizó seguimiento anestésico estándar y anestesia. Antes y después de la cirugía, se realizó la medición ONSD para seguir los cambios en la PIC. La medición ONSD se puede utilizar como un método eficaz, no invasivo, repetido y fácil de aplicar para monitorear los cambios en la PIC en pacientes pediátricos con SC que se planea someter a craniectomía.

Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis: a single center experience.

INTRODUCTION: Management of ventriculomegaly in pediatric patients with syndromic craniosynostosis (SC) requires understanding the underlying mechanisms that cause increased intracranial pressure (ICP) and the role of cerebrospinal fluid (CSF) in cranial vault expansion in order to select the best treatment option for each individual patient. METHODS: A total of 33 pediatric patients with SC requiring craniofacial surgery were retrospectively evaluated. Cases of nonsyndromic craniosynostosis and shunt-induced craniosynostosis were excluded. Six syndrome-based categories were distinguished: Crouzon syndrome, Pfeiffer syndrome, Apert syndrome, cloverleaf skull syndrome, and others (Muenke syndrome, Sensenbrenner syndrome, unclassified). All of the patients were treated surgically for their cranial deformity between 2010 and 2016. The presence of ventriculomegaly and ventriculoperitoneal (VP) shunt requirement with its impact in cranial vault expansion were analyzed. Clinical and neuroimaging studies covering the time from presentation through the follow-up period were revised. The mean postoperative follow-up was 6 years and 3 months. A systematic review of the literature was conducted through a PubMed search. RESULTS: Of the total of 33 patients with SC, 18 (54.5%) developed ventriculomegaly and 13 (39.4%) required ventriculoperitoneal (VP) shunt placement. Six patients (18.2%) required shunt placement previous to craniofacial surgery. Seven patients (21.2%) required a shunt after craniofacial surgery. Seven fixed pressure ventriculoperitoneal shunts and six programmable valves were placed as first choice. All patients improved their clinical symptoms after shunt placement. Aesthetic results seemed to be better in patients with programmable shunts. CONCLUSIONS: Unless clear criteria for overt hydrocephalus are present, it is recommended to perform craniofacial surgery as a first step in the management of patients with SC in order to preserve the expansive effect of CSF for cranial vault expansion. In our experience, the use of externally programmable valves allows for the treatment of hydrocephalus while maintaining the expansive effect of CSF for the remodeling of the cranial vault. Prospective evaluations are needed to determine causality.

Short-term survival in extensive craniofacial resections

OBJECTIVES: Craniofacial resection (CFR) procedures for craniofacial tumors with cranial extension are often extensive. Although CFRs may yield good oncological results, there are concerns about high perioperative morbidity and mortality. This study aimed to determine risk factors for perioperative mortality after open CFR in terms of deaths occurring during index hospitalizations. METHODS: We conducted a retrospective analysis of CFRs conducted at a tertiary oncology hospital from May 2009 through December 2018. RESULTS: Our analysis included data from the medical records of 102 patients, the majority of whom were male (n=74, 72.5%). The mean age was 61 years (±18.3 years). Skin malignancies (n=64, 63.4%) accounted for nearly two-thirds of the treated tumors, and most of these were squamous cell carcinoma. Postoperative medical complications occurred in 33 patients (33%), and surgical complications occurred in 48 (47%). Multivariate analysis revealed the only independent risk factors for perioperative deaths to be the presence of intracranial tumor extension on preoperative imaging (hazard ratio [HR]=4.56; 95% confidence interval [CI]: 1.74-11.97; p=0.002) and the unexpected emergence of postoperative neurological dysfunction (HR=10.9; 95% CI: 2.21-54.3; p=0.003). CONCLUSIONS: In our study, factors related to tumor extension were associated with a higher risk of perioperative death.

Fibrous dysplasia with secondary aneurysmal bone cyst-a rare case report and literature review.

INTRODUCTION: Fibrous dysplasia (FD) and aneurysmal bone cyst (ABC) are uncommon non-neoplastic intraosseous lesions, and the occurrence of concomitant FD and ABC is extremely rare. CASE REPORT: An 11-year-old boy presented with gradual progressive enlargement of his right zygomatic bone over 4 years prior to presentation. Computed tomography revealed a lesion with a central bony area showing a ground-glass appearance surrounded by a well-defined expansile lesion with internal septations. An incisional biopsy was performed and suggested a fibro-osseous lesion. Resection was performed, followed by immediate reconstruction using autogenous bone graft from the iliac crest. Histopathological examination revealed irregularly shaped trabeculae comprising immature woven bone in a fibroblastic cell-rich stroma. Blood-filled sinusoidal spaces lined by fibrous septa containing scattered multinucleated giant cells were observed peripherally. These findings were compatible with concomitant FD and ABC. The patient was disease-free at the time of his 10-month follow-up. CONCLUSIONS: Based on our literature search, this report is the first to describe concomitant monostotic FD and ABC in the zygomatic bone. Accurate diagnosis requires careful investigation and examination of clinical, radiographical, and histopathological features. The treatment of choice should provide an esthetic and functional improvement in the patient.

Craneosinostosis sindrómica: Características craneodentofaciales, tratamiento ortodón-tico-quirúrgico y factores asociados a tipos de síndrome / Craniossinostose sindrômica: Características craniodentofaciais, tratamento ortodônti-co-cirúrgico e fatores associados aos tipos de síndrome / Syndromic Craniosynostosis: Craniodentofacial features, orthodontic-surgical treatment and factors associated to types of syndrome

Objetivos: Describir las características craneodentofaciales, tratamientos ortodónticos-quirúrgicos y establecer una aso-ciación entre los tipos de síndrome presentes en pacientes con craneosinostosis sindrómica (CS). Material y métodos: Estudio retrospectivo de registros médicos y de ortodoncia de pacientes con CS. Los datos se recogieron en una forma específica y se sometieron a un análisis estadístico descriptivo para observar la distribución de frecuencias y se utilizó la prueba de Chi cuadrado con un nivel de significación del 5% para asociar el síndrome y los tipos de variables. Resultados: El síndrome de Crouzon fue el tipo predominante (59.2%) y la braquicefalia fue el tipo más frecuente de craneosinostosis (63.6%). Hubo una asociación significativa entre las variables braquicefalia (p = 0,014), presencia de paladar hendido (p = 0,043), mordida cruzada posterior (p = 0,013), distracción osteogénica realizada por elásticos intermaxilares (p = 0,030), barra de Erich (p = 0.007) y la extracción (p = 0.041) y los síndromes estudiados. Conclusión: Los pacientes con CS a menudo tienen cambios craneodentofaciais y algunas variables tienen asociaciones significativas en relación con los tipos de síndromes.

Objectives: Describe the craniodentofacial characteristics, orthodontic-surgical treatments and establish an association between the syndrome types present in patients with syndromic craniosynostosis (SC). Material and methods: Retros-pective study of medical and orthodontic records of patients with SC. Data was collected on a specific form and subjected to descriptive statistical analysis to observe the distribution of frequencies and chi-square test with level of significance of 5% was used to associate syndrome and the types of variables. Results: Crouzon syndrome was the predominant type (59.2%) and brachycephalia was the most prevalent type of craniosynostosis (63.6%). There was a significant association between the variables brachycephaly (p=0.014), presence of cleft palate (p=0.043), posterior cross bite (p=0.013), distrac-tion osteogenesis performed by intermaxillary elastics (p= 0.030), Erich bar (p= 0.007) and extraction (p=0.041) and the syndromes studied. Conclusion: Patients with SC often have craniodentofaciais changes and some variables had signifi-cant associations in relation to the types of syndromes.

Descrever as características craniodentofaciais, tratamentos ortodôntico-cirúrgicos e estabelecer uma associação entre os tipos de síndrome presentes em pacientes com craniossinostose sindrômica (CS). Material e métodos: Estudo retrospectivo de prontuários médicos e ortodônticos de pacientes com CS. Os dados foram coletados em formulário específico e submetidos à análise estatística descritiva para observar a distribuição das frequências e o teste qui-quadrado com nível de significância de 5% foi utilizado para associar a síndrome e os tipos de variáveis. Resultados: A síndrome de Crouzon foi o tipo predominante (59,2%) e a braquicefalia foi o tipo mais prevalente de craniossinostose (63,6%). Houve associação significativa entre as variáveis braquicefalia (p = 0,014), presença de fissura de palato (p = 0,043), mordida cruzada posterior (p = 0,013), distração osteogênica realizada pelos elásticos intermaxilares (p = 0,030), barra de Erich (p= 0,007) e extração (p = 0,041) e as síndromes estudadas. Conclusão: Pacientes com CS frequentemente apresentam alterações craniodentofaciais e algumas variáveis apresentaram associações significativas em relação aos tipos de síndromes.

Técnica de fixação com fio metálico associado ao uso de resina acrílica, para redução de fratura no corpo da mandíbula em cão – relato de caso / Metallic wire fixation technique associated with the use of acrylic resin to reduce fracture in the jaw body in dogs – case report

As fraturas de mandíbula são bastante frequentes na clínica de pequenos animais, sendo importante para o Médico Veterinário saber diagnosticar e tratar adequadamente os pacientes que chegam ao hospital; muitas vezes, em estado crítico. O método de diagnóstico utilizado amplamente, ainda hoje, é a radiografia, por ser de baixo custo e, consequentemente, acessível para a maior parte da população, além de proporcionar diagnósticos confiáveis. A escolha do tipo de tratamento (conservativo ou cirúrgico) vai depender basicamente do tipo de fratura e de como o cirurgião vai proceder, analisando o escore de avaliação da fratura. É imprescindível, portanto, que as técnicas conservadoras e cirúrgicas, disponíveis para a realização do procedimento terapêutico, devam ser conhecidas pelo Médico Veterinário, para a correta execução durante o procedimento. O objetivo desse trabalho foi relatar um caso de redução de fratura no corpo da mandíbula de um cão, utilizando a técnica de fixação com fio metálico, associado ao uso de resina acrílica. A técnica associada, utilizada nesse trabalho, apresentou-se adequada, demonstrando resultados positivos para o tipo de paciente que estava sendo tratado, animal jovem, agitado e que a tutora não tinha tempo disponível para, regularmente, oferecer a atenção necessária aos cuidados da fratura do animal.

Mandibular fractures are quite common in small animals. Therefore, it is important for the veterinarian how to diagnose and treat, with an adequate approach, the patients that arrive at the hospital often in a critical condition, requiring a proper stabilization and then undergo with the necessary procedures to increase the chances of animal survival and reduce the possibility of sequel. Even today, the method of diagnosis widely used is the x-ray, since it is considered to have a low cost and then affordable for the majority of the people. The type of treatment (conservative or surgical) depends largely on fracture and how a surgeon wants to proceed, analyzing the factors related to the evaluation fracture score. It is imperative that the Veterinarian has total dominion over the cranial anatomy. Moreover, the surgeon has to know all the techniques available to carry out the therapeutic procedures with conservative or surgical techniques. The goal of this study was to report a case of fracture reduction in the mandibular body of a dog using the fixation technique with wire, associated with the use of acrylic resin. The associated technique used in this study can be considered as satisfactory, for showing positive results for the type of patient who was being treated, taking into account the mechanical, biological and clinical factors.

Técnica de fixação com fio metálico associado ao uso de resina acrílica, para redução de fratura no corpo da mandíbula em cão relato de caso / Metallic wire fixation technique associated with the use of acrylic resin to reduce fracture in the jaw body in dogs case report

As fraturas de mandíbula são bastante frequentes na clínica de pequenos animais, sendo importante para o Médico Veterinário saber diagnosticar e tratar adequadamente os pacientes que chegam ao hospital; muitas vezes, em estado crítico. O método de diagnóstico utilizado amplamente, ainda hoje, é a radiografia, por ser de baixo custo e, consequentemente, acessível para a maior parte da população, além de proporcionar diagnósticos confiáveis. A escolha do tipo de tratamento (conservativo ou cirúrgico) vai depender basicamente do tipo de fratura e de como o cirurgião vai proceder, analisando o escore de avaliação da fratura. É imprescindível, portanto, que as técnicas conservadoras e cirúrgicas, disponíveis para a realização do procedimento terapêutico, devam ser conhecidas pelo Médico Veterinário, para a correta execução durante o procedimento. O objetivo desse trabalho foi relatar um caso de redução de fratura no corpo da mandíbula de um cão, utilizando a técnica de fixação com fio metálico, associado ao uso de resina acrílica. A técnica associada, utilizada nesse trabalho, apresentou-se adequada, demonstrando resultados positivos para o tipo de paciente que estava sendo tratado, animal jovem, agitado e que a tutora não tinha tempo disponível para, regularmente, oferecer a atenção necessária aos cuidados da fratura do animal.(AU)

Mandibular fractures are quite common in small animals. Therefore, it is important for the veterinarian how to diagnose and treat, with an adequate approach, the patients that arrive at the hospital often in a critical condition, requiring a proper stabilization and then undergo with the necessary procedures to increase the chances of animal survival and reduce the possibility of sequel. Even today, the method of diagnosis widely used is the x-ray, since it is considered to have a low cost and then affordable for the majority of the people. The type of treatment (conservative or surgical) depends largely on fracture and how a surgeon wants to proceed, analyzing the factors related to the evaluation fracture score. It is imperative that the Veterinarian has total dominion over the cranial anatomy. Moreover, the surgeon has to know all the techniques available to carry out the therapeutic procedures with conservative or surgical techniques. The goal of this study was to report a case of fracture reduction in the mandibular body of a dog using the fixation technique with wire, associated with the use of acrylic resin. The associated technique used in this study can be considered as satisfactory, for showing positive results for the type of patient who was being treated, taking into account the mechanical, biological and clinical factors.(AU)

Utilización de células troncales en reconstrucción cráneo-maxilo-facial en humanos / Stem cells in human cranio-maxillo-facial reconstruction

El propósito de este estudio fue evaluar la evidencia en relación al empleo de técnicas reconstructivas en el área cráneo-facial asociado el uso de células troncales en humanos. Se realizó una revisión sistemática de la literatura en las bases Medline, ScienceDirect, EMBASE, TripDatabase, LILACS entre marzo del 2004 a marzo del 2016 con criterios de elegibilidad y estrategia de búsqueda definida. La selección de artículos fue realizada por dos investigadores de forma independiente y cuando ellos presentaron discordancia, un tercer investigador realizó le selección. Se encontraron un total de 382 artículos, se realizó una selección de artículos, eliminado duplicados, estudios experimentales en animales y selección según análisis de resúmenes, se seleccionaros 14 artículos con procedimientos reconstructivos en el área cráneo-facial asociado a células troncales. Existe limitada evidencia de calidad en relación a la utilización de células troncales en reconstrucción cráneo facial en humanos. A excepción de un estudio (ensayo clínico de bajo nivel de evidencia), todos corresponden a series o reporte de casos, con lo cual no es posible recomendar su utilización en procedimientos reconstructivos. Es necesario realizar estudios de evidencia sustentable con el empleo de células troncales que permitan identificar su real rendimiento al compararlo con otras técnicas quirúrgicas.

The purpose of this study was to evaluate the evidence regarding the use of reconstructive techniques in the craniofacial area associated with the use of stem cells in humans. A systematic review of the literature was conducted in the databases Medline, ScienceDirect, EMBASE, TripDatabase, LILACS between March 2004 and March 2016 with eligibility criteria and defined search strategy. The selection of articles was done by two researchers independently and when they presented discordance, a third researcher made the selection. We found a total of 382 articles, made a selection of articles, removed duplicates, experimental studies in animals and selection according to analysis of abstracts, we selected 14 articles with reconstructive procedures in the craniofacial area associated with stem cells. There is limited quality evidence regarding the use of stem cells in the craniofacial reconstruction in humans. With the exception of one study (clinical trial of low level of evidence), all correspond to series or report of cases, with which it is not possible to recommend its use in reconstructive procedures. It is necessary to carry out studies of sustainable evidence with the use of stem cells that allow to identify its real performance when compared with other surgical techniques.

Aportes y modificaciones de técnicas quirúrgicas en cirugía craneofacial pediátrica / Contributions and modifications of surgical techniques in pediatric craniofacial surgery

Las deformidades craneofaciales se deben en su mayoría a alteraciones del crecimiento y desarrollo, traumatismos y neoplasias. Con el objetivo de describir el tratamiento quirúrgico integral de estas y las modificaciones y aportes realizados a las técnicas craneofaciales tradicionales, se realizó un estudio descriptivo transversal en 46 pacientes tratados por el equipo interdisciplinario de cirugía craneofacial del Hospital Pediátrico Universitario Juan M Márquez, en el período comprendido entre mayo de 2003 a marzo de 2007. La edad promedio de los pacientes intervenidos fue de 6,3 años. Se realizó una veintena de técnicas quirúrgicas. La más utilizada fue la suturectomía coronal con hemiavance en antifaz y craneoplastia frontal, para el 15,2 por ciento; seguida de la suturectomía coronal con avance en antifaz y craneoplastia frontal, para el 10,9 por ciento. El 8,9 por ciento de los pacientes necesitó osteotomía Le Fort III para distracción del tercio medio. Los aportes introducidos fueron: la plicatura radiada de la duramadre con bipolar en el 75,0 por ciento de los craneotomizados, incisión puntiforme de la duramadre para descomprimir hipertensión encefálica transoperatoria o retraer encéfalo para osteotomías de base de cráneo en el 64,3 por ciento de los craneotomizados; fractura en tallo verde a nivel del pterium, en el colgajo en antifaz para las técnicas de Marshall, usada en el 83,3 por ciento de los braquicéfalos o plagiocéfalos; el avance sólo del lado afectado en el 100 por ciento de las plagiocefalias, con modificación de la técnica de Marshall, entre otras, para un total de 9 modificaciones a los procederes quirúrgicos. Hubo ausencia de complicaciones asociadas a éstas, con más de 2 años de evolución posoperatoria(AU)

Most of he craniofacial deformities are caused by development and growth alterations, traumata and neoplasms. To describe the integral surgical treatment of above deformities and the modifications and contributions carried out to traditional craniofacial techniques, a cross-sectional and descriptive study was conducted in 46 patients seen by the craniofacial surgery multidisciplinary staff from Juán Manuel Máquez University Children Hospital from May, 2003 to March, 2007. Mean age of the patients operated on was of 6.3 years. Twenty Surgical tehniques were performed where the more used was he coronal suturectomy with a mask hemi-advance and frontal cranioplasty for the 15.2 percent, followed by the coronal suturectomy with the two above techniques for te 10.9 percent. The 8.9 percent of patients need LeFort III osteotomy for distraction of half-third. The contributions included: radiate plication of dura mater with the bipolar type in the 75,0 percent of craniotomy patients, punctiform incision of dura mater to decompress a transoperative encephalic hypertension or to retract the encephalon for osteotomies on skull base in the 64,3 percent of craniotomy patients, greenstick fracture at level of pterion in the mask flap for the Marshal's techniques used in the 83,3 percent of the brachycephalous or plagiocephaly with advance only of inolved side in the 100 percent of plagiocephalies with a modification of Marshall's technique among others, for a total o 9 modifications to surgical procedures. There weren't complications associated with these procedures and more than two years of posoperative cours(AU)

Aportes y modificaciones de técnicas quirúrgicas en cirugía craneofacial pediátrica / Contributions and modifications of surgical techniques in pediatric craniofacial surgery

Las deformidades craneofaciales se deben en su mayoría a alteraciones del crecimiento y desarrollo, traumatismos y neoplasias. Con el objetivo de describir el tratamiento quirúrgico integral de estas y las modificaciones y aportes realizados a las técnicas craneofaciales tradicionales, se realizó un estudio descriptivo transversal en 46 pacientes tratados por el equipo interdisciplinario de cirugía craneofacial del Hospital Pediátrico Universitario Juan M Márquez, en el período comprendido entre mayo de 2003 a marzo de 2007. La edad promedio de los pacientes intervenidos fue de 6,3 años. Se realizó una veintena de técnicas quirúrgicas. La más utilizada fue la suturectomía coronal con hemiavance en antifaz y craneoplastia frontal, para el 15,2 por ciento; seguida de la suturectomía coronal con avance en antifaz y craneoplastia frontal, para el 10,9 por ciento. El 8,9 por ciento de los pacientes necesitó osteotomía Le Fort III para distracción del tercio medio. Los aportes introducidos fueron: la plicatura radiada de la duramadre con bipolar en el 75,0 por ciento de los craneotomizados, incisión puntiforme de la duramadre para descomprimir hipertensión encefálica transoperatoria o retraer encéfalo para osteotomías de base de cráneo en el 64,3 por ciento de los craneotomizados; fractura en tallo verde a nivel del pterium, en el colgajo en antifaz para las técnicas de Marshall, usada en el 83,3 por ciento de los braquicéfalos o plagiocéfalos; el avance sólo del lado afectado en el 100 por ciento de las plagiocefalias, con modificación de la técnica de Marshall, entre otras, para un total de 9 modificaciones a los procederes quirúrgicos. Hubo ausencia de complicaciones asociadas a éstas, con más de 2 años de evolución posoperatoria(AU)

Most of he craniofacial deformities are caused by development and growth alterations, traumata and neoplasms. To describe the integral surgical treatment of above deformities and the modifications and contributions carried out to traditional craniofacial techniques, a cross-sectional and descriptive study was conducted in 46 patients seen by the craniofacial surgery multidisciplinary staff from Juán Manuel Máquez University Children Hospital from May, 2003 to March, 2007. Mean age of the patients operated on was of 6.3 years. Twenty Surgical tehniques were performed where the more used was he coronal suturectomy with a mask hemi-advance and frontal cranioplasty for the 15.2 percent, followed by the coronal suturectomy with the two above techniques for te 10.9 percent. The 8.9 percent of patients need LeFort III osteotomy for distraction of half-third. The contributions included: radiate plication of dura mater with the bipolar type in the 75,0 percent of craniotomy patients, punctiform incision of dura mater to decompress a transoperative encephalic hypertension or to retract the encephalon for osteotomies on skull base in the 64,3 percent of craniotomy patients, greenstick fracture at level of pterion in the mask flap for the Marshal's techniques used in the 83,3 percent of the brachycephalous or plagiocephaly with advance only of inolved side in the 100 percent of plagiocephalies with a modification of Marshall's technique among others, for a total o 9 modifications to surgical procedures. There weren't complications associated with these procedures and more than two years of posoperative cours(AU)

Estesioneurablastoma y panhipopituitarismo: presentación de un caso / Esthesionerublastoma and panhypopituitarysm: case report

Objetivos: presentar un caso de estesioneuroblastoma que desarrolla hipopituitarismo como complicación grave de su tratamiento. Realizar una revisión de la literatura reciente sobre el tratamiento y pronóstico de estos tumores. Presentar una sistemática de pesquisa de hipipituitarismo en estos pacientes. Descripción: paciente masculino de 68 años que se presenta con metástasis submaxilar de estesioneuroblastoma. La RMN mostraba una masa neoplásica de gran tamaño centrada sobre el etmoides, que comprometía base de cráneo y corteza fronto-orbitaria izquierda. Intervención: se realizó tratamiento combinado de radio y quimioterapia preoperatorios, y cirugía por vía craniofacial. A los 20 meses ingresa con un cuadro convulsivo e hiponatremia grave. Se hace diagnóstico de panhipopituitarismo y el cuadro revierte con la administración de hidrocortisona de 10 mg vía oral. Conclusión: el estesioneuroblastoma es un tumor infrecuente cuyo tratamiento incluye cirugía, radioterapia y quimioterapia. Es necesario un alto índice de sospecha y el seguimiento de por vida para pesquisar la instalación del hipopituitarismo como complicación grave de su tratamiento.

Objectives. To report a case of esthesioneuroblastoma associated to hypopituitarism as a late complication of its treatment, to review the literature regarding treatment and prognosis and to resent a guideline for the diagnosis of hypopituitarism in these patients. Description. A 68-year-old male patient presented with a submaxilar metastasis of a very large esthesioneuroblastoma that involved both ethmoidal bones and invaded left cribiform plate and frontal lobe. Intervention. The patient underwent combined modality treatment with preoperative radio and chemotherapy, and craniofacial sugery of the tumor. Twenty months later he was admitted with a seizure and severe hyponatremia. A diagnosis of panhypopituitarism was made, and his sodium reverted to normal with de administration of 10 mg of hydrocortisone p.o. Conclusions Esthesioneuroblastomas are rare tumours that require multimodality treatment with surgery, radiotherapy and chemotherapy. Hypopituitarism is a late and potentially fatal complication that must be suspected and tested during the patients‘ whole lifespan.

Equipo cubano interdisciplinario de cirugía craneofacial en pediatría: Resultados de un quinquenio / Interdisciplinary Cuban staff of craniofacial surgery in Pediatrics: Results from a five-year period

Las deformidades craneofaciales se deben en su mayoría a alteraciones del crecimiento y desarrollo, traumatismos y neoplasias. Con el objetivo de describir el tratamiento quirúrgico integral de estas, se realizó un estudio descriptivo transversal en 46 pacientes tratados por el Equipo Intertidisciplinario de Cirugía Craneofacial del Hospital Pediátrico Universitario Juan M. Márquez en el periodo comprendido entre mayo de 2003 a marzo de 2007. La deformidad más frecuente fue la craneosinostosis (57 por ciento), específicamente la plagiocefalia (17 por ciento). La mayoría de los pacientes fueron de piel blanca (70 por ciento) y se intervinieron entre el 1ro.y 7mo.años de vida (57 por ciento), con una media de 6,3 años. En general no hubo predilección por sexo. Se emplearon 18 diferentes técnicas quirúrgicas. Se presentaron cinco complicaciones durante el posoperatorio (11 por ciento), una defunción (2,2 por ciento), una oftalmoplejía (2,2 por ciento), dos salidas de líquido cefalorraquídeo (4,4 por ciento) y una desaturación por depresión del centro respiratorio (2,2 por ciento). Excepto la oftalmoplejía hubo resolución de las complicaciones en un intervalo de 15 días. Los resultados reportados por este equipo, único de su tipo en el país, son relevantes al compararlos con la literatura internacional(AU)

Most of the craniofacial deformities are due to alterations of growth, development, traumata and neoplasms. To describe the integral surgical treatment of above deformities, a cross-sectional and descriptive study was conducted in 46 patients treated by the interdisciplinary staff of craniofacial surgery from the Juan Manuel Márquez University Children Hospital from May, 2003 to March, 2007. The more frequent deformity was the craniosynostosis (57 percent), specifically the plagiocephaly (17 percent). Most of patients were of white race (70 percent) and were operated don between the first and the seventh year of life (57 percent) for a mean of 6,3 years. Generally there wasn't sex predominance. A total of 18 different surgical techniques were used. There were 5 complications during the postoperative period (11 percent), a decease (2.2 percent), a ophthalmoplegia (2.2 percent), two cerebrospinal fluid leakage (4.4 percent) and a desaturation by depression of respiratory center (2.2 percent). With the exception of ophthalmoplegia there was a resolution of complications in a 15 days interval. Results reported by this unique Cuban staff are outstanding compared with international literature(AU)

Equipo cubano interdisciplinario de cirugía craneofacial en pediatría: Resultados de un quinquenio / Interdisciplinary Cuban staff of craniofacial surgery in Pediatrics: Results from a five-year period

Las deformidades craneofaciales se deben en su mayoría a alteraciones del crecimiento y desarrollo, traumatismos y neoplasias. Con el objetivo de describir el tratamiento quirúrgico integral de estas, se realizó un estudio descriptivo transversal en 46 pacientes tratados por el Equipo Intertidisciplinario de Cirugía Craneofacial del Hospital Pediátrico Universitario Juan M. Márquez en el periodo comprendido entre mayo de 2003 a marzo de 2007. La deformidad más frecuente fue la craneosinostosis (57 por ciento), específicamente la plagiocefalia (17 por ciento). La mayoría de los pacientes fueron de piel blanca (70 por ciento) y se intervinieron entre el 1ro.y 7mo.años de vida (57 por ciento), con una media de 6,3 años. En general no hubo predilección por sexo. Se emplearon 18 diferentes técnicas quirúrgicas. Se presentaron cinco complicaciones durante el posoperatorio (11 por ciento), una defunción (2,2 por ciento), una oftalmoplejía (2,2 por ciento), dos salidas de líquido cefalorraquídeo (4,4 por ciento) y una desaturación por depresión del centro respiratorio (2,2 por ciento). Excepto la oftalmoplejía hubo resolución de las complicaciones en un intervalo de 15 días. Los resultados reportados por este equipo, único de su tipo en el país, son relevantes al compararlos con la literatura internacional

Most of the craniofacial deformities are due to alterations of growth, development, traumata and neoplasms. To describe the integral surgical treatment of above deformities, a cross-sectional and descriptive study was conducted in 46 patients treated by the interdisciplinary staff of craniofacial surgery from the Juan Manuel Márquez University Children Hospital from May, 2003 to March, 2007. The more frequent deformity was the craniosynostosis (57 percent), specifically the plagiocephaly (17 percent). Most of patients were of white race (70 percent) and were operated don between the first and the seventh year of life (57 percent) for a mean of 6,3 years. Generally there wasn't sex predominance. A total of 18 different surgical techniques were used. There were 5 complications during the postoperative period (11 percent), a decease (2.2 percent), a ophthalmoplegia (2.2 percent), two cerebrospinal fluid leakage (4.4 percent) and a desaturation by depression of respiratory center (2.2 percent). With the exception of ophthalmoplegia there was a resolution of complications in a 15 days interval. Results reported by this unique Cuban staff are outstanding compared with international literature

Evaluation of porous vitreous carbon or silicon implants by radiology in rat's skull / Avaliação radiológica de implantes de carbono vítreo poroso ou silicone em crânio de ratos

PURPOSE: Evaluate by CT the use of porous vitreous carbon (PVC) and silicon (S) implants as the replacement bone in the craniofacial skeleton of rats. METHODS: 40 rats divided in: Group A (n=20) PVC submitted to the implant of a fragment in skull. After the euthanasia, the animals were divided into two subgroups: A I: 10 animals, studied in the 7th postoperative day (P.O) and AII: 10 animals, studied in the 28th P.O. In group B, S, 20 rats were submitted to S implant in the skull. All other steps were identical to group A, with designation of subgroups BI and BII. CT with beams in axial cuts of 1 mm thickness to obtain 3-D information It was used Hounsfield scale for evaluate the radio density of the implant. They were used non parametric tests to analyze the results. RESULTS: The 7th PO boss remained in the two groups, but for 28th PO, observed reduction in the volume of the implant in Group A, not observed in group B. CT studies noticed different radio densities around all of S prostheses (pseudo-capsule), that don't appeared in CPV implants. The S has remained unchanged in the CT, but the CPV has had a modification in its radio density (p<0,05), in all implants. CONCLUSION: In CT evaluation the implants of CPV have greater deformation that the S, which makes them not suitable for replacement of membranous bone in the rat skull.

OBJETIVO: Realizar avaliação através de tomografia computadorizada (TC) de implantes de carbono vítreo poroso (CVP) e silicone (S) para sua utilização na substituição óssea no esqueleto craniofacial de ratos. MÉTODOS: Foram utilizados 40 ratos Wistar divididos em: Grupo A (n=20), implantes subperiostais de CVP no crânio. Após o momento da eutanásia os animais foram divididos em dois subgrupos: A I: 10 animais, estudados no 7(0) dia pós-operatório (PO) e AII: 10 animais, estudados no 28(0) PO. No grupo B (n=20), os ratos foram submetidos ao implante de silicone no crânio. Todas outras etapas foram idênticas ao grupo A, com a designação de subgrupos BI e BII. Foi realizada tomografia computadorizada com cortes axiais de 1 mm de espessura para obtenção de imagens tridimensionais. A escala de Hounsfield foi utilizada para avaliação da radiodensidade dos implantes. Testes estatísticos não paramétricos foram utilizados para analisar os resultados. RESULTADOS: O volume do implante foi mantido ao 7(0) PO nos dois grupos, mas ao 28(0) PO, ocorreu uma redução no volume do implante no grupo A, não observada no grupo B. Os estudos tomográficos demonstraram a presença de uma pseudo-cápsula ao redor dos implantes no grupo B, não observada nos implantes de CVP. Os implantes de silicone permaneceram inalterados na TC, mas os de CVP apresentaram modificação na sua radiodensidade e deformação (p<0,05). CONCLUSÃO: Na avaliação, através de TC, os implantes de CPV apresentam maior deformação que os de S, o que os torna inadequados para substituição do osso membranoso no crânio de ratos.

Complicações e fatores prognósticos em 175 ressecções craniofaciais de tumores malígnos da base anterior do crânio / Complications and prognostic factors in 175 craniofacial resections for malignant tumors of the anterior skull base

Introdução: A ressecção radical de tumores malígnos originários na base do crânio permanece um desafio, principalmente pelo risco de complicações graves. Embora vários fatores prognósticos estejam bem estabelecidos, a maioria das informações foi baseada em séries de casos retrospectivas e heterogêneas. Objetivo: analisar as taxas de complicação e os fatores prognósticos de uma série de pacientes submetidos à ressecção craniofacial de tumores malignos da base anterior do crânio na instituição. Pacientes e Método: Foram revisados os prontuários de 175 pacientes tratados entre 1955 e 1998 na instituição, incluindo pacientes com tumores envolvendo a base anterior do crânio que necessitaram exposição ou ressecção de dura-máter ou nasofaringe, independente de tratamento prévio. Resultados: Os tumores localizavam-se nos seios paranasais (75), órbita (58), cavidade nasal (26) e nasofaringe (16). A maioria era carcinomas espinocelulares (91 casos). As margens cirúrgicas foram negativas em 104 pacientes (60%), exíguas em 18 (10%) e positivas em 53 (30%). Complicações cirúrgicas ocorreram em 39 pacientes (22,3%), sendo infecção/deiscência em 12,1% dos casos e fístula liqüórica em 13,4%. A sobrevida atuarial em 5 anos foi de 57%. A análise multivariada usando o modelo de regressão de Cox identificou que a infiltração de dura-máter, margens positivas, tipo histológico, tratamento oncológico prévio e metástases cervicais foram fatores prognósticos independentes neste estudo. Conclusão: A ressecção craniofacial permite a remoção em bloco de diversos tumores da base anterior do crânio, com morbidade aceitável e significante impacto na sobrevida desses pacientes.

Background: Radical resection of malignant tumors arising in the skull base remains a challenge because of the risk of serious complications. Although several prognostic factors are well established, the available information is most based on heterogeneous retrospective case series. Objective: to analyze complication rates and prognostic factors in a large series of patients submitted to anterior craniofacial resection in a single institution. Materials and methods: We reviewed 175 patients treated from 1955 to 1998, including patients with involvement of the anterior skull base that required exposure or resection of the dura mater or the nasopharynx, independently of previous treatment. Results: The tumor sites were: paranasal sinuses (75), orbit (58), nasal cavity (26), and nasopharynx (16). Most tumors were squamous cell carcinomas (91 cases). Surgical margins were negative in 104 patients (60%), close in 18 (10%) and positive in 53 (30%). Surgical complications occurred in 39 patients (22.3%) (soft tissue infection/dehiscence, 12.1%; cerebrospinal fluid leak, 13.4%). The 5-year actuarial overall survival was 57%. Multivariate analysis using Cox regression model identified infiltration of dura mater, positive margins, histological type, prior oncological treatment, and neck metastasis as independent prognostic predictors. Conclusion: Craniofacial oncologic resection allows en bloc removal of several tumors of the anterior skull base, with acceptable morbidity and significant impact on survival of these patients.