Manejo de vía aérea en paciente con leontiasis ossea. Reporte de caso / Airway management in a patient with leontiasis ossea. Case report

Leontiasis ossea is an uncommon complication of advanced chronic kidney disease that alters the facial bone and the airway, making its perioperative management more complex. We present a clinical case of a female with Leontiasis ossea presenting a difficult airway which requires parathyroidectomy. Assessment, planning and management of the airway by awake intubation is described.

La leontiasis ossea es una complicación infrecuente de la enfermedad renal crónica avanzada que altera el macizo facial óseo y la vía aérea, complejizando su manejo perioperatorio. Presentamos caso clínico de mujer portadora de leontiasis ossea con vía aérea difícil requiriendo paratiroidectomía. Se describe valoración, planificación y manejo de vía aérea mediante intubación vigil.

Frontal Sinus Osteoma Accompanied by Intracranial Mucocele and Local Hyperostosis Frontalis Interna.

Frontal sinus osteoma accompanied by intracranial mucocele and local hyperostosis frontalis interna has never been reported. A 47-year-old woman presented with a 3-month history of intermittent headache. Physical examination revealed no neurologic abnormality. Contrasted magnetic resonance imaging showed a frontal heterogeneously enhanced lesion with adjacent nonenhanced cyst. Computed tomography showed a bone density mass, which was accompanied by local hyperostosis frontalis interna, which filled the left frontal sinus and extended intracranially. The patient underwent a left frontobasal craniotomy. Both the osseous mass and cyst capsule were removed totally via a frontal craniotomy, followed by skull base reconstruction. The postoperative course was uneventful. The final pathologic diagnosis was osteoma and mucocele.

Uremic Leontiasis Ossea in a Patient With Chronic Renal Insufficiency Demonstrated on Bone Scintigraphy.

A 37-year-old woman with chronic renal insufficiency underwent bone scintigraphy to evaluate renal osteodystrophy (ROD). Markedly increased uptakes were shown in the maxilla and the mandible, which suggested extensive maxillary and mandibular hypertrophy. CT image revealed that diffuse bony thickening and ground-glass appearance in the skull, maxilla, and mandible with poor distinction of the corticomedullary junction. Whole-body bone scintigraphy images also demonstrated various skeletal characteristics of ROD. This case emphasizes the utility of bone scintigraphy for the surveillance of the whole body in ROD.

Hiperostosis frontal interna y meningiomas múltiples como hallazgos autópsicos incidentales. Aportación de un caso y revisión de la literatura / Hyperostosis frontalis interna and multiple meningiomas as incidental autopsy findings. A case report and review of the literature

La hiperostosis frontal interna es una entidad caracterizada por un engrosamiento óseo desmesurado de la tabla interna del hueso frontal, de carácter benigno. A pesar de que fue ampliamente discutida en el pasado, esta entidad rara vez se menciona en la literatura patológica actual. Afecta predominantemente a mujeres posmenopáusicas y suele ser un hallazgo casual durante la realización de pruebas de imagen o de autopsias. Aunque fue descrita hace más 300 años, su prevalencia se ha visto incrementada desde finales del siglo XX, indicando un cambio profundo en la fertilidad humana con la introducción de tratamientos hormonales y nuevos hábitos alimentarios. Presentamos un caso de hiperostosis frontal interna y meningiomas múltiples en una mujer posmenopáusica pluripatológica, que se consideraron hallazgos autópsicos incidentales no relacionados con la causa de su muerte (AU)

Hyperostosis frontalis interna is a benign condition characterized by excessive bone thickening of the inner table of the frontal bone. Although it was widely discussed in the past, this entity is now rarely mentioned in the literature. It predominantly affects postmenopausal women and is usually an incidental finding during testing imaging or autopsy. It was described three centuries ago but since the late 20th century it has been more frequent, reflecting a profound change in life style, especially with regards to fertility and the introduction of hormonal treatments, as well as new eating habits. We present a case of hyperostosis frontalis interna and multiple meningiomas, incidental autopsy findings in a postmenopausal woman with multiple disease, and unrelated to the cause of death (AU)

[Hyperostosis frontalis interna and epilepsy]. / Hyperostosis frontalis interna et épilepsie.

We have analysed the clinical history of a 48-year old female patient who has been admitted to hospital after an epileptic fit. The tomodensitometry examination has shown a hyperostosis frontalis interna. This condition is frequent and is characterized by a progressive thickening of the frontal bone internal table. The aetiology is unknown; the most probable hypothesis is a deterioration of the androgen-estrogen ratio. This woman suffers from epileptic fits for 40 years now but also from hereditary polycystic kidney disease and CREST syndrome. In this analysis, we have applied the principle of parcimony and we have examined the role played by the sexual hormones in these pathologies.

Bases para la terapia con líquidos y electrólitos. Modelos fisiológicos del equilibrio ácido-base (II): los nuevos modelos. Parte 1 / Basis for fluid and electrolyte therapy. Physiological mod­els of acid-base balance (II): the new models. Part 1

En las últimas dos décadas hemos asistido a una revolución en el conocimiento científico de la fisiología y las alteraciones del equilibrio ácido-base. En la primera parte de esta serie de artículos revisamos el modelo «tradicional», la aproximación centrada en el bicarbonato y basada en el trabajo pionero de Henderson y Halsselbalch, que es aún la más utilizada en la práctica clínica diaria. En la segunda y la tercera parte revisamos la teoría de otros modelos más modernos, particularmente el de Stewart, derivado al final de los años setenta desde las leyes de la química física. Con este modelo, tal como fue desarrollado por Peter Stewart y Peter Constable, utilizando la presión parcial de dióxido de carbono (pCO2), la diferencia de iones fuertes (SID) y la concentración total de ácidos débiles ([Atot]), somos capaces de predecir con exactitud la acidez del plasma y deducir el saldo neto de iones no medidos (NUI). La interpretación del equilibrio ácido-base no será nunca más un arte intuitivo y arcano. Se ha convertido en un cálculo exacto que puede realizarse automáticamente con ayuda del software moderno. En las últimas tres partes, utilizando a pie de cama el strong ion calculator y la historia clínica, mostraremos cómo el modelo fisicoquímico cuantitativo tiene ventajas sobre los tradicionales, principalmente en las situaciones fisiológicas extremas que se viven con los pacientes de la unidad de cuidados intensivos pediátrica o en las alteraciones congénitas del metabolismo (AU)

A revolution has recently undergone in the last two decades in the scientific understanding of acid-base physiology and dysfunction. In the first part of this series we review the "traditional" model, the current bicarbonate-centered approach based on the pioneering work of Henderson and Halsselbalch, still the most widely used in clinical practice. In the second and third part we review theoretically other modern approaches, particularly Stewart's one, derived in the late 1970s from the laws of physical chemistry. Whit this approach, as developed by Peter Stewart and Peter Constable, using the partial pressure of carbon dioxide (pCO2), the strong ion difference (SID) and the concentration of weak acids ([Atot]) we can now predict accurately the acidity of plasma and deduce the net concentration of unmeasured ions (NUI). Acid-base interpretation has ceased to be an intuitive an arcane art and became an exact computation that can be automated with modern software. In the last three parts, using at the bedside the quantitative Strong Ion Calculator together with the medical history, we show how quantitative acid-base analysis has advantages over traditional approaches, mainly in the extreme physiological situations of clinical scenarios like the paediatric intensive care unit or the congenital metabolic diseases (AU)

Causa inusual de dificultad respiratoria: hernia de Morgagni asociada con el síndrome de Down / Unusual cause of respiratory distress: Morgagni hernia associated to Down’s syndrome

La hernia de Morgagni (HM) es una hernia diafragmática poco frecuente, con un índice de hernias diafragmáticas congénitas del 2%. El síndrome de Down (SD) es la anomalía cromosómica más frecuente notificada. Desde el punto de vista clínico, hay una variación muy amplia entre individuos por sus rasgos característicos y las malformaciones sistémicas asociadas. La coexistencia de hernia de Morgagni y síndrome de Down se ha notificado en aproximadamente un 20%. En el servicio de urgencias se ingresó a una mujer con SD para evaluar una neumonía recurrente acompañada de fiebre y tos seca persistente. En este caso, se observó que la anomalía radiográfica era en realidad HM, con asas intestinales en el lado derecho del tórax. Presentamos este caso para dejar constancia de una asociación asintomática entre el SD y la HM (AU)

Morgagni hernia (MH) is a rare diaphragmatic hernia with 2% rate of congenital diaphragmatic hernias. Is reported Down’s syndrome (DS) the most common chromosomal anomaly. There is a wide range variation among individuals clinically by its characteristic features and associated systemic malformations. The coexistence of Morgagni hernia and DS is reported approximately 20% . A female patient with DS was admitted to emergency department for evaluation of recurrent pneumonia accompanied by persistent dry cough and fever. In the case the radiographic abnormality was actually found to be MH with intestinal loops in the right thorax. We report this case to notify an asymptomatic association between DS and MH (AU)

Full penetrance of Morgagni-Stewart-Morel syndrome in a 75-year-old woman: case report and review of the literature.

CONTEXT: Morgagni-Stewart-Morel syndrome is defined as the presence of hyperostosis frontalis interna, variably associated with metabolic, endocrine, and neuropsychiatric disorders. The possible cause-effect relationship of these associations remains uncertain. CASE PRESENTATION: A 75-year-old woman presented with severe frontal headache and a history of psychotic disorders. On instrumental examination she was found to have extensive frontal hyperostosis and cortical atrophy. These findings, associated to the metabolic and neuropsychiatric pattern of the patient, are consistent with a high penetrance of Morgagni-Stewart-Morel syndrome. EVIDENCE ACQUISITION AND SYNTHESIS: In this clinical case seminar, we summarize the current understanding of the association between hyperostosis frontalis interna and Morgagni-Stewart-Morel, based on a MEDLINE search (case reports, original articles, and reviews published between 1928 and 2011) on this topic. Possible pathophysiological mechanisms underlying both the headache and the hyperostosis frontalis interna are discussed. CONCLUSION: A case of full penetrance of Morgagni-Stewart-Morel syndrome is reported, presenting many of the clinical features described in the literature. Metabolic and endocrine dysfunctions should be interpreted not only as isolated components of the syndrome, but also as the reason behind its pathogenesis. Endocrine or nutritional disorders may have led to an altered bone metabolism with frontal bone apposition. On the other hand, the severity of our patient's neurological and psychiatric symptoms correlates well with the severity of her hyperostosis frontalis interna and the cortical atrophy.

Frontal cortex dysfunction due to extensive hyperostosis frontalis interna.

An 87-year-old patient was found to have an unusually protrusive hyperostosis frontalis interna, discovered on MRI examination during an assessment of cognitive decline. Neuropsychological evaluation suggested direct repercussions of the frontal lobe compression on executive functions, as well as psychiatric disorders and possibly memory loss.

Hyperostosis frontalis interna in a patient with giant cell arteritis.

Hyperostosis frontalis interna (HFI) is a disorder characterized by progressive symmetric thickening of the inner table of the frontal bone of the human skull. HFI may be accompanied by headache and some neuropsychiatric diseases such as epilepsy and dementia. Giant cell arteritis (GCA), also called temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries. It affects elderly people and may result in a wide variety of systemic, neurologic and ophthalmologic complications. As no association of HFI and GCA was encountered in the literature, we found it interesting to report a case with both of these clinical entities.

Headache in a patient with Klinefelter's syndrome and hyperostosis frontalis interna.

Hyperostosis frontalis interna (HFI) has been reported in older women, but reports in men are rare. We present a novel case of migraine headache in a gentleman with Klinefelter's syndrome and HFI, along with a discussion of possible pathophysiologic mechanisms underlying both the headache and the HFI.

Angiosarcoma sobre linfedema crónico / Angiosarcoma in chronic lymphedema

El angiosarcoma que se desarrolla sobre una extremidad con linfedema crónico se denomina síndrome de Stewart-Treves. Éste aparece típicamente como una complicación de un linfedema de larga evolución localizado en el brazo, tras mastectomía y/o radioterapia por un cáncer de mama. Existen casos de síndrome de Stewart-Treves sobre linfedema crónico en la extremidad superior contralateral al cáncer de mama tratado y sobre linfedema crónico de pierna. Presentamos dos casos de este síndrome. El primero corresponde a un típico síndrome de Stewart-Treves en una mujer de 83 años, que fue diagnosticada de angiosarcoma en el territorio de un linfedema crónico secundario a mastectomía y radioterapia por un cáncer de mama. El segundo caso es mucho más raro, ya que se trata de un caso de angiosarcoma difuso de pierna, en un hombre de 42 años e historia de linfedema. La naturaleza agresiva de este síndrome precisa de su conocimiento e investigación de tratamientos para prevenirlo

Angiosarcoma that develops on a limb with chronic lymphedema is called Stewart-Treves syndrome. This typically appears as a complication of a long course lymphedema located on the arm, after mastectomy and/or radiotherapy due to breast cancer. There are cases of Stewart-Treves syndrome in chronic lymphedema in the upper limb contralateral to the breast treated for cancer and in chronic lymphedema of the leg. We present two cases of this syndrome. The first corresponds to a typical syndrome of Stewart-Treves in an 83-year-old woman who was diagnosed of angiosarcoma in a chronic lymphedema territory secondary to mastectomy and radiotherapy due to breast cancer. The second case is much rarer, since it is a case of diffuse angiosarcoma of the leg in a 42-year-old man with a history of lymphedema. Due to the aggressive nature of this syndrome, knowledge and research on its treatment are necessary