RESUMEN
Introducción: Los craneofaringiomas son malformaciones histológicamente benignas que se sitúan entre el hipotálamo y la hipófisis, zonas con un rol determinante en la modulación de la saciedad. Aun siendo tumores benignos, presentan una considerable morbilidad. La obesidad está presente hasta en un 52% de los pacientes. Objetivo: evaluar factores de riesgo cardiovascular, composición corporal y gasto energético en pacientes con craneofaringioma, y compararlos con un grupo de obesos multifactoriales. Material y métodos: Se incluyeron todos los pacientes con resección quirúrgica de craneofaringioma, menores de 21 años, en seguimiento en nuestro centro entre mayo 2012 hasta abril 2013 que aceptaron participar por medio del consentimiento informado. Se realizó valoración antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta y valoración de ingesta energética y de macronutrientes. Se determinó resistencia a la insulina (HOMA-IR) y dislipemia. Se comparó a los pacientes con craneofaringioma con obesidad, con un grupo de pacientes con obesidad multifactorial. Resultados: se estudiaron 39 pacientes. El 59% era obeso y presentó significativamente menor% de masa magra (62.4 vs 67.5 p=0.01) y mayor% de masa grasa (37.5 vs 32.5 p=0.01) comparados con los obesos multifactoriales. No se encontró diferencias en el compromiso metabólico entre los obesos con y sin antecedente de craneofaringioma. Se dividieron los pacientes en tertilos según% de gasto energético para categorizar en gasto bajo vs normal. Se encontró asociación positiva entre% de gasto energético y% de masa magra en obesos multifactoriales (68±1%; en los gasto normal vs 62.6± 1% en los gasto bajo: p 0,04). Sin diferencias dentro de la población de obesos con antecedente de craneofaringioma (62±2.7 en los gasto normal/alto vs 61.2±1.8% en los gasto bajo: p 0,8). El gasto energético basal (REE) fue menor en los pacientes con antecedente de craneofaringioma vs obesos multifactoriales, independientemente de la masa magra, lo que sustenta que existirían otros factores que actuarían disminuyendo el gasto energético. No hubo diferencia con respecto a la ingesta en ambos grupos estudiados. Conclusiones: los pacientes con antecedente de craneofaringioma presentan menor gasto energético no relacionado a la masa magra y similar ingesta energética comparado con obesos multifactoriales. No hubo diferencias en el compromiso metabólico entre los obesos con y sin antecedentes de craneofaringioma (AU)
Introduction: Craniopharyngiomas are histologically benign malformations located between hypothalamus and the pituitary gland, areas that play an important role in satiety modulation. Although the tumors are benign, they may cause significant morbidity. Obesity is found in up to 52% of patients. Aim: To assess cardiovascular risk factors, body composition, and energy expenditure in patients with craniopharyngioma, and to compare them to results in a group of children with multifactorial obesity. Material and methods: All patients who underwent surgical resection of craniopharyngioma, younger than 21 years of age, who were being followed-up at our center between May 2012 and April 2013 who gave their informed consent to participate were enrolled in the study. Anthropometric measurements, body composition with impedanciometer, energy expenditure with indirect calorimetry, and energy and macronutrient intake were evaluated. Insulin resistance (HOMA-IR) and dyslipidemia were determined. Patients with craniopharyngioma associated with obesity were compared to patients with multifactorial obesity. Results: Of 39 patients studied, 59% were obese and a significantly lower percentage of lean mass (62.4 vs 67.5 p=0.01) and a higher percentage of fat mass (37.5 vs 32.5 p=0.01) compared to multifactorial obese subjects. No differences were found in metabolic involvement between obese subjects with and those without a history of craniopharyngioma. Patients were divided into tertiles according to percentage of energy expenditure to categorize low versus normal expenditure. A positive correlation was found between percentage of energy expenditure and lean mass percentage in subjects with multifactorial obesity (68±1%; in those with normal energy expenditure versus 62.6±1% in those with low energy expenditure: p 0.04). No difference was found within the group of obese patients with a history of craniopharyngioma (62±2.7 in those with normal/high expenditure versus 61.2±1.8% in those with low expenditure: p 0.8). Baseline energy expenditure (BEE) was lower in craniopharyngioma patients than in those with multifactorial obesity, regardless of lean mass percentage, supporting the hypothesis that other factors may be involved in the decrease of energy expenditure. There was no difference in the food intake between both groups. Conclusions: Patients with a history of craniopharyngioma had a lower energy expenditure unrelated to lean mass and a similar energy intake compared to subjects with multifactorial obesity. No differences were found in metabolic involvement between obese subject with and those without a history of craniopharyngioma (AU)
Asunto(s)
Humanos , Preescolar , Niño , Adolescente , Composición Corporal/fisiología , Craneofaringioma/metabolismo , Ingestión de Energía/fisiología , Enfermedades Metabólicas/metabolismo , Obesidad/metabolismo , Neoplasias Hipofisarias/metabolismo , Craneofaringioma/complicaciones , Estudios Transversales , Enfermedades Metabólicas/complicaciones , Obesidad/complicaciones , Estudios Observacionales como Asunto , Neoplasias Hipofisarias/complicaciones , Estudios ProspectivosRESUMEN
La craneoestenosis es una anomalía congénita en la cual una o más suturas se fusionan prematuramente, generando una forma anormal del cráneo. Sin tratamiento, puede producir hipertensión endocraneana, pérdida visual, epilepsia y retraso madurativo además del compromiso estético. Su resolución es quirúrgica, en lo posible dentro de los primeros meses de vida. La recuperación posquirúrgica inmediata se realiza habitualmente en unidades de cuidados intensivos (UCI). El objetivo del presente estudio es analizar mediante una evaluación prospectiva y longitudinal la atención de pacientes operados de craneoestenosis en una unidad de cuidados intermedios y moderados (CIM). Se elaboró un protocolo de atención y se capacitó al personal médico y de enfermería. Criterios de inclusión: posquirúrgico de craneoestenosis simple, edad menor de 36 meses, cumplimiento de un período de estabilización hemodinámica en sala de recuperación anestésica. Criterios de exclusión: inestabilidad hemodinámica, y/o respiratoria, complicaciones intra quirurgicas, alto requerimiento transfusional intra quirúrgico, arritmias. Se analizaron los resultados mediante el programa Epi info 6.0. Cumplieron los criterios de inclusión 44 pacientes, mediana de edad 9 meses (rango 3 menos 36). Los diagnósticos más frecuentes fueron escafocefalia n=31p y plagiocefalia n=9p. El procedimiento quirúrgico más utilizado fue la sagitectomía n=27p. La complicación más frecuente fue anemia (98 por ciento de los pacientes). Otras complicaciones: colección hemática subgaleal (11.4 por ciento), dolor (9 por ciento), hipotermia y acidosis metábolica (9 por ciento). No hubo muertes ni infecciones del sistema nervioso central. La estadia media fue de 4 dias. Conclusiones: Mediante la modalidad implementada, los pacientes que cursan el posquirúrgico de craneoestenosis pueden ser atendidos en CIM, permitiendo la internación conjunta con los padres y optimizando la utilizacion de plazas de UCI
Asunto(s)
Lactante , Preescolar , Constricción Patológica , Cráneo , Cuidados Críticos , Cuidados Posoperatorios , Suturas Craneales , Estudios LongitudinalesRESUMEN
Massive osteolysis (MO) is a rare condition in which progressive localized bone tissue resorption is associated with proliferating thin-walled vessels in the absence of inflammation. Rare cases have been reported to occur in the skull. This paper describes two patients with MO who presented with massive assymetric swelling of the skull. This was associated with extensive enlargement of the paranasal sinuses (frontal, ethmoidal, and sphenoidal in one and the mastoid air cells in the other). The second patient developed subcutaneous emphysema on several occasions and the Valsalva maneuver increased the swelling, indicating transmission of the air from the nasopharynx to the mastoid cells and from there to the subcutaneous tissue. In the first patient, the sinus mucosa was shown to be involved by an extensive lymphangioma, and a similar change was seen in the mastoid air cells (patient 2). We are proposing that MO of these two patients resulted from bone resorption due to progressive extension of sinus mucous lymphangiomata.
Asunto(s)
Linfangioma/complicaciones , Apófisis Mastoides/patología , Osteólisis Esencial/etiología , Neoplasias de los Senos Paranasales/complicaciones , Senos Paranasales/patología , Cráneo/patología , Adolescente , Femenino , Humanos , Linfangioma/diagnóstico por imagen , Linfangioma/patología , Masculino , Apófisis Mastoides/diagnóstico por imagen , Osteólisis Esencial/diagnóstico por imagen , Osteólisis Esencial/patología , Neoplasias de los Senos Paranasales/diagnóstico por imagen , Neoplasias de los Senos Paranasales/patología , Senos Paranasales/diagnóstico por imagen , Cráneo/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
Bone distraction of the superior and medial thirds of the craniofacial skeleton en bloc, avoiding a frontal craniectomy is presented. We applied this procedure in eight patients who were more than 5 years old with different types of craniofacial synostosis and who had not received previous treatment, and with a normal frontal shape. During monobloc advancement, major complications were encountered in older patients, especially the impossibility of the brain to expand rapidly to fill the retrofrontal dead space. Distraction osteogenesis of the craniofacial skeleton en bloc (without craniectomy) is feasible. Miniplates and screws are avoided as well as the possibility of frontal relapse or fractures of the frontozygomatic region. The patients did not need skull vault remodeling, except for a small cranioplasty at the bregma zone. The results obtained were satisfactory and stable at the time. This procedure avoids any kind of osteosynthesis, there is no extradural dead space, the operative time is brief, and blood loss is minimal. The inconvenience is the necessity of a second operation to remove the distractor.
Asunto(s)
Acrocefalosindactilia/cirugía , Disostosis Craneofacial/cirugía , Huesos Faciales/cirugía , Osteogénesis por Distracción/métodos , Cráneo/cirugía , Pérdida de Sangre Quirúrgica , Encéfalo/patología , Niño , Preescolar , Suturas Craneales/cirugía , Craneotomía/métodos , Femenino , Estudios de Seguimiento , Hueso Frontal/cirugía , Humanos , Masculino , Osteogénesis por Distracción/efectos adversos , Osteogénesis por Distracción/instrumentación , Hueso Parietal/cirugía , Factores de Tiempo , Resultado del Tratamiento , Cigoma/cirugíaRESUMEN
A series of 54 patients with lateral ventricle tumors diagnosed and surgically treated from 1988 to 1998 was reviewed. Neoplasms invading ventricles and originating beyond their walls were excluded. There were 35 male and 19 female patients. Their ages ranged from 15 days to 20 years, and two frequency peaks were observed, one at 2 and one at 11 years. The most frequent signs and symptoms were attributed to increased intracranial pressure. The 54 patients included 41 who developed hydrocephalus, but only 15 of these required shunting. The trigonal region and frontal horn were the most common sites of origin. Surgery was planned with due consideration for the localization of the tumor, its presumptive histology, its main feeding vessels, the parenchymal functionality, and the presence or absence of hydrocephalus. The most frequent tumor types were subependymal giant cell astrocytoma, choroid plexus tumors, ependymoma, and astrocytoma. The most common complications were intraventricular hemorrhage, cortical collapse, subdural collection and seizures. To conclude, tumors located within the lateral ventricles are often very voluminous and are predominantly benign, and the treatment of choice is total resection. In the case of malignancy, postsurgical radiotherapy and/or chemotherapy should be given.
Asunto(s)
Neoplasias del Ventrículo Cerebral/diagnóstico , Neoplasias del Ventrículo Cerebral/terapia , Ventrículos Laterales , Procedimientos Neuroquirúrgicos/métodos , Adolescente , Adulto , Neoplasias del Ventrículo Cerebral/complicaciones , Niño , Preescolar , Femenino , Glioma/complicaciones , Glioma/diagnóstico , Glioma/terapia , Humanos , Lactante , Recién Nacido , Ventrículos Laterales/patología , Ventrículos Laterales/cirugía , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Análisis de Supervivencia , Tomografía Computarizada por Rayos XRESUMEN
Up to a few years ago, patients with cloverleaf skull deformity underwent partial surgery to relieve intracranial hypertension with poor functional and aesthetic results, often leading to relapses and reoperations, both in our own experience and in that of other authors. As of 1990, however, we started to use the technique described by Persing et al. to resolve the complex cloverleaf malformation in a single definitive procedure. Five patients, whose ages ranged from 2 months to 5 years, achieved satisfactory results both as regards relief from intracranial hypertension and preservation of visual acuity, and from the aesthetic viewpoint. Surgical approaches and their modifications are described.
Asunto(s)
Craneosinostosis/cirugía , Craneotomía/métodos , Cefalometría , Craneosinostosis/diagnóstico por imagen , Femenino , Humanos , Hidrocefalia/cirugía , Procesamiento de Imagen Asistido por Computador , Lactante , Masculino , Posición Prona , Seudotumor Cerebral/diagnóstico por imagen , Seudotumor Cerebral/cirugía , Reoperación , Tomografía Computarizada por Rayos XRESUMEN
Forty-eight consecutive children treated for craniopharyngioma at the Juan P. Garrahan National Paediatric Hospital (Buenos Aires, Argentina) from 1988 to 1994 are described. Complications of patients undergoing total resection alone and those undergoing subtotal or partial resection plus radiotherapy were compared. Survival time and quality of life proved more satisfactory in the former group, as there were no recurrences. In contrast, among the latter patients, 53% suffered relapses. Endocrinological complications were similar in the two groups. Postsurgical subdural haematomas were quite frequent and eight patients required treatment for intracranial hypertension. Vascular complications, though less common, led to high morbidity and mortality. There was a considerable incidence of shunt malfunction (80%), arguing against placement of a preoperative shunt, which tended besides to foster postsurgical subdural haematomas.