Resección transoral de la apófisis estiloides por síndrome de Eagle: caso clínico / Transoral resection of the stiloyd process for Eagle síndrome: case report

El síndrome de Eagle está caracterizado por una elongación o una curvatura medial excesiva de la apófisis estiloides o por una calcificación del ligamento estilohioideo que puede provocar dolor cervicofacial o síntomas neurológicos por la compresión de los vasos o nervios del cuello. El tratamiento más eficaz es el quirúrgico y consiste en la resección de la apófisis estiloides; puede ser realizado por vía externa o mediante un abordaje transoral. Se describe el caso clínico de un paciente con síndrome de Eagle que fue tratado con éxito mediante un abordaje transoral, sin amigdalectomía y con asistencia de endoscopios. (AU)

Eagle syndrome is characterized by an elongation or excessive medial curvature of the styloid process or calcification of the stylohyoid ligament that can cause cervicofacial pain or neurological symptoms due to compression of the vessels or nerves of the neck. The most effective treatment is surgical and consists of resection of the styloid process, it can be performed by externally or through a transoral approach.The clinical case of a patient with Eagle syndrome who was successfully treated by a transoral approach, without tonsillectomy and with the assistance of endoscopes, is described. (AU)

Osificación heterotópica de cadera asociada a pancreatitis aguda severa: reporte de un caso / Hip heterotopic ossification associated with severe acute pancreatitis: a case report

La osificación heterotópica (OH), es la formación anormal de hueso maduro dentro de tejidos blandos extra esqueléticos donde normalmente no existe tejido óseo. Varias formas de OH han sido descritas de acuerdo a su presentación clínica, localización y ocurrencia progresiva o aislada. Su presentación en pacientes sometidos a inmovilización prolongada en el contexto de coma farmacológicamente inducido, en ausencia de lesiones del sistema nervioso central, es poco habitual. Presentamos el caso de una paciente de 40 años, sexo femenino, sin antecedentes mórbidos, que presenta episodio de pancreatitis aguda grave, manejada en UCI. Producto de lo anterior, requiere coma farmacológicamente inducido por 3 meses y hospitalizaciones reiteradas y prolongadas durante los 10 meses siguientes. Durante 3 años desde la resolución de su cuadro inicial evoluciona con alteración progresiva de la marcha y rigidez de la extremidad inferior derecha, sin eventos traumáticos durante ese período. Se objetiva mediante radiografía y TAC foco de OH coxofemoral derecha. Se resuelve de forma quirúrgica y biopsia de pieza operatoria confirma el diagnóstico. La paciente logra buena recuperación posterior. NIVEL DE EVIDENCIA: IV

Heterotopic ossification (HO) is the abnormal formation of mature bone within extraskeletal soft tissues where bone does not exist. Various presentation of HO have been described according to the clinical settings and location of the lesions, and progressive or isolated occurrence. A rare form of presentation occurs in induced coma patients with long-term immobilization and without central nervous system injuries. We present the case of a 40 years old female patient, without previous morbidity, who develop a severe acute pancreatitis. The patient requires an intensive care unit management (ICU) and a 3-month pharmacology induced coma and reiterative and prolonged hospitalizations during the next 8 months. During 3 years after resolution of her base disease, patient develops a progressive step claudication and a hip rigidity in adduction and external rotation. A coxofemoral HO focus is confirmed by radiology and CT. A surgical treatment of HO was performed, and the initial diagnose confirmed by anatomic pathology after biopsy of the injury. Patient had a favorable outcome.

Fibrodisplasia osificante progresiva plus por una variante patogénica del gen ACVR1: caso clínico / Fibrodysplasia ossificans progressiva: report of one case

Fibrodysplasia ossificans progressiva (FOP) or myositis ossificans, is a genetic disease, with a prevalence of 1 in 2.000.000. It is caused by pathogenic variants in ACVR1 gene and characterized by soft tissue heterotopic ossification, starting in the second decade of life. It is associated to early mortality caused by respiratory complications. It evolves in flare-ups, triggered by soft tissue injuries; therapy is symptomatic, using analgesia, steroids and diphosphonates. We report a 12-year-old female with left renal agenesis, hallux valgus and intellectual disability, presenting with a six months history of thoracic kyphosis, tender nodules in the thorax, and rigidity of right elbow and left knee. Clinical examination revealed dysmorphic facial features. A magnetic resonance showed heterotopic ossification nodules, which was confirmed with spinal radiography. These findings prompted the diagnosis of FOP. Pain treatment was started, and prednisone was used during flare-ups. The ACVR1 gene was analyzed and a pathogenic variant, p. Arg206His, was found, confirming the diagnosis of FOP.

Hemosiderosis pulmonar idiopática con osificación pulmonar / Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification

La osificación pulmonar es un hallazgo poco usual, generalmente asintomático, que se reporta como incidental en biopsias de pulmón. Asimismo, la hemosiderosis pulmonar idiopática es una causa poco frecuente de infiltración pulmonar. Se presenta el caso de un hombre de 64 años con síntomas respiratorios crónicos, en quien se detectaron estas dos condiciones en el estudio histopatológico.

Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged

Cuadro sincopal en el contexto del síndrome de Eagle / Episode of syncope in Eagles syndrome

El síndrome de Eagle es una patología infrecuente, caracterizada por la presencia de dolor cervicofacial, cefalea y calambres que se proyectan en la garganta, en el cuello y en el oído. Describimos el caso de una mujer de 36 años, con historia de tumefacción cervical, sensación de irritación faríngea y síncope, en la que en los estudios radiológicos se encontró una osificación total de ambos ligamentos estilohioideo, causante de su cuadro sincopal por compresión carotídea.

Eagle's syndrome is an infrequent pathology characterized by the presence of cervicofacial pain, headache and cramps that are projected in the throat, neck and ear. We reported a case of a 36 year-old woman with a history of cervical swelling, sore throat and syncope, which on imaging studies was found ossification both stylohyoid ligaments, causing carotid compression and syncope.

Osificación del ligamento estilohioideo en 3028 radiografías panorámicas digitales / Ossification of the stylohyoid ligament in 3028 digital panoramic radiographs

La mayoría de los diagnósticos de procesos estiloides elongados y osificaciones de ligamentos estilohioideos corresponden a hallazgos en el análisis de radiografías panorámicas. En la presente investigación se evaluaron 3028 Radiografías Panorámicas digitales, tomadas entre Junio de 2009 y Junio de 2011. Se registraron las osificaciones de ligamento estilohiodeo de acuerdo a sexo, edad y bilateralidad. El análisis de datos se desarrolló con el Test estadistico Chi cuadrado, con un nivel de significancia estadística a< 0,05. En las imágenes evaluadas se encontró una prevalencia de osificaciones de un 8,42%, siendo más frecuentes en individuos de sexo femenino, osificaciones bilaterales y con patrón discontinuo. En cuanto a la edad se evaluó de acuerdo a grupos de edad menor o igual a 40 años y mayores de 40 años, encontrándose relación con la variable patrón de osificación. También se encontró relación entre las variables lateralidad y patrón de osificación. El presente estudio entrega información relevante para la odontología y la otorrinolaringología.

Most diagnoses of elongated styloid processes and ossification of stylohyoideus ligaments correspond to findings in the analysis of panoramic radiographs. In the present study we evaluated 3028 digital panoramic radiographs, taken between June 2009 and June 2011. Stylohyoideus ligament ossifications were recorded according to sex, age and bilaterality. Data analysis was carried out with the chi square test, with a statistical significance level a <0.05. In the images assessed we found a prevalence of 8.42% of ossifications, which was more frequent in female subjects, and bilateral ossification discontinuous pattern. In terms of age we evaluated according to age group less than or equal to 40 and older than 40 years, as related to the variable pattern of ossification. We also found relation between laterality variables and patterns of ossification. This study provides information relevant to dentistry and otolaryngology.

Radiographic study of ossification of the pterygospinous and pterygoalar ligaments by the hirtz axial technique

The correct radiographic identification of ossification of the pterygospinous and pterygoalar ligaments plays an important role in surgical procedures for the treatment of trigeminal neuralgia. Most of these procedures are performed through the foramen ovale, a site where these ligaments can be found to be partially or completely ossified. We studied the radiographic features of these ossified ligaments and their location in relation to the foramen ovale by the Hirtz axial technique. For this purpose, 93 dry skulls from the Discipline of Anatomy, Sao Jose dos Campos Dental School, UNESP, which presented partial or complete ossification of these ligaments, were radiographed. The pterygospinous ligament was detected on 27.97% of radiographs and was partially ossified in 19.36% of cases and completely ossified in 8.61%. The pterygoalar ligament was present in 62.35% of radiographs, being partially ossified in 49.44% and completely ossified in 12.91%. The pterygospinous ligaments was found to be partially and completely ossified on the same radiograph in 3.23% of cases, whereas the pterygoalar ligament appeared partially and completely ossified on the same radiograph in 6.45%. Furthermore, the pterygospinous ligament was thinner than the pterygoalar ligament and located more medially in relation to the foramen ovale. The pterygoalar ligament formed a large bone bar lateral to the foramen ovale, often obliterating the lumen of the latter. The Hirtz axial technique is an excellent tool for the observation of complete or partial ossification of the pterygospinous and pterygoalar ligaments in surgical procedures for the treatment of trigeminal neuralgia performed through the foramen ovale.

A correta identificacao radiografica da ossificacao dos ligamentos pterigoespinhoso e pterigoalar e de grande importancia nos procedimentos cirurgicos no tratamento da nevralgia trigeminal. A maioria desses procedimentos e feita via forame oval, local onde podemos encontrar esses ligamentos ossificados, parcial ou totalmente. Estudamos, pela tecnica axial de Hirtz, as caracteristicas radiograficas desses ligamentos ossificados e sua localizacao em relacao ao forame oval. Para isso foram radiografados 93 cranios secos, pertencentes a Disciplina de Anatomia da Faculdade de Odontologia de Sao Jose dos Campos - UNESP, que apresentavam a ossificacao parcial ou total dos referidos ligamentos. Encontramos o ligamento pterigoespinhoso em 27,97% das radiografias, sendo parcialmente ossificado em 19,36% e totalmente em 8,61%. O ligamento pterigoalar estava presente em 62,35% das radiografias, estando parcialmente ossificado em 49,44% e totalmente em 12,91%. O ligamento pterigoespinhoso foi encontrado parcial e totalmente ossificado, numa mesma radiografia, em 3,23% dos casos, enquanto o ligamento pterigoalar apareceu parcial e totalmente ossificado, na mesma radiografia, em 6,45% dos casos. Observamos ainda que o ligamento pterigoespinhoso era menos espesso, em relacao ao ligamento pterigoalar, e localizado mais para medial, em relacao ao forame oval, enquanto que o ligamento pterigoalar formava uma larga barra ossea lateralmente ao forame oval, obliterando muitas vezes o lumen do mesmo. A tecnica axial de Hirtz e um excelente meio para a observacao da ossificacao total ou parcial destes ligamentos, quando de procedimentos cirurgicos que utilizam o forame oval para o tratamento da nevralgia trigeminal.

Surgery for dural ossification in association with cervical ossification of the posterior longitudinal ligament via an anterior approach.

STUDY DESIGN: Direct removal of an ossified mass via an anterior approach carries good decompression, to one- or two-level ossification of the posterior longitudinal ligament (OPLL) of the cervical spine. Ossification occasionally involves not only the posterior longitudinal ligament (PLL) but also the underlying dura mater. Defect of the dura mater by resection of the dural ossification (DO) can cause cerebrospinal fluid leakage or neural injury. The technique of resection of OPLL with floating of DO provides satisfactory decompression and avoids dural defect or neural injury in OPLL associated with DO. METHODS: Four patients developed cervical myelopathy. Radiological examination revealed cord compression due to OPLL associated with DO. RESULTS: All patients underwent anterior procedures. After the necessary discectomies and corpectomies, OPLL was resected using a high-speed drill with a 4-mm steel burr and then with a 4-mm diamond burr. When the OPLL became paper-thin, it was separated from the dura mater using a microdissector and a Kerrison rongeur. There was a thin layer of the nonossified degenerated PLL between the residual OPLL and DO. Meticulous dissection of the residual OPLL over the DO was performed without removing the DO at this layer. Fixation was performed with a titanium cylindrical cage. CONCLUSION: This technical note describes the successful decompression of the spinal cord by removing OPLL only, and avoidance of dural defect or neural injury in cases of OPLL associated with DO.