Imaging in the study of macrocephaly: Why?, when?, how?

Macrocephaly is a clinical term defined as an occipitofrontal circumference more than two standard deviations above the mean. It is present in 5% of children and is a common indication for imaging studies. There are multiple causes of macrocephaly; most of them are benign. Nevertheless, in some cases, macrocephaly is the clinical manifestation of a condition that requires timely medical and/or surgical treatment. The importance of imaging studies lies in identifying the patients who would benefit from treatment. Children with macrocephaly associated with neurologic alterations, neurocutaneous stigmata, delayed development, or rapid increase of the circumference have a greater risk of having disease. By contrast, parental macrocephaly is predictive of a benign condition. Limiting imaging studies to patients with increased risk makes it possible to optimize resources and reduce unnecessary exposure to tests.

Radiología en el estudio de la macrocefalia. ¿Por qué?, ¿cuándo?, ¿cómo? / Imaging in the study of macrocephaly: Why?, when?, how?

Macrocefalia es un término clínico definido como el incremento de la circunferencia occipitofrontal por encima de dos desviaciones estándar. Se presenta en el 5% de los niños y es una indicación frecuente de estudios radiológicos. Existen múltiples causas de macrocefalia, que corresponden mayoritariamente a condiciones benignas. Sin embargo, en algunos casos es la manifestación clínica de una patología que requiere una oportuna intervención médico-quirúrgica. La relevancia del estudio radiológico radica en la identificación de estos pacientes. Aquellos niños que se presentan con macrocefalia asociada a alteraciones neurológicas, estigmas neurocutáneos, retraso del desarrollo o rápido aumento de la circunferencia craneal poseen un riesgo aumentado de presentar patología. Por el contrario, el antecedente de macrocefalia parenteral es predictivo de una condición benigna. Acotar el estudio radiológico a los pacientes de mayor riesgo permite optimizar recursos y disminuir la exposición innecesaria a exámenes.(AU)

Macrocephaly is a clinical term defined as an occipitofrontal circumference more than two standard deviations above the mean. It is present in 5% of children and is a common indication for imaging studies. There are multiple causes of macrocephaly; most of them are benign. Nevertheless, in some cases, macrocephaly is the clinical manifestation of a condition that requires timely medical and/or surgical treatment. The importance of imaging studies lies in identifying the patients who would benefit from treatment. Children with macrocephaly associated with neurologic alterations, neurocutaneous stigmata, delayed development, or rapid increase of the circumference have a greater risk of having disease. By contrast, parental macrocephaly is predictive of a benign condition. Limiting imaging studies to patients with increased risk makes it possible to optimize resources and reduce unnecessary exposure to tests.(AU)

Intra-arterial cisplatin given prior to surgery in osteosarcoma: grade of necrosis and size of tumor as major prognostic factors.

From January 1983 to August 1987, 29 evaluable patients with high-grade osteosarcoma were treated in our institution with preoperative intra-atrial cisplatin, 100 mg/m2 every 14 days for three courses. Surgery was done on day 42. Surgery consisted of limb salvage in six, amputations in 15, and disarticulations in eight. Postoperative chemotherapy included Adriamycin (ADR), 45 mg/m2 for 2 days every 6 weeks, alternated with cisplatin 120 mg/m2 every 6 weeks. The nephrotoxicity (18 out 29) was reversible in all cases. Cardiotoxicity was prominent; it was observed in 31% of patients. In six, there was congestive heart failure, but there were no fatal cases. The hematological toxicity was severe. There were three patients with fatal infections who had no evidence of disease after they had finished treatment. Seventeen of 29 patients (58.6%) were good responders and showed 60-100% tumoral necrosis after intra-atrial cisplatin. The 6-year, relapse-free survival rate was 58.6%--70.5% for the good responders and 41.6% for the poor responders (p less than 0.05). The size of the tumor was the other important prognostic factor. The rate of 6-year, relapse-free survival was 73.6% for small tumors (those measuring less than 100 cm2) and 33.3% for large tumors (p less than 0.05).