RESUMEN
The Yunis-Varon syndrome is a rare hereditary disorder with ectodermal and skeletal anomalies that include agenesis or hypoplasia of the clavicles, craniofacial disproportion with severe micrognathia, digital anomalies, prenatal and postnatal growth deficiency, and neonatal death. Only 12 cases have been reported in the literature. Although neonatal death is a significant feature of this syndrome, two case reports describe children, both males, who were 30 months and 3 years of age at the time of investigation. The 30-month-old child was reexamined at 11 years of age, and had further clinical and radiologic features that included hypodontia, impacted permanent teeth, spinal defects, cardiomegaly, bilateral hearing loss, and metatarsus adductus. Children who survive the neonatal period and continue to thrive with many of the features of the Yunis-Varon syndrome as well as the new features described in this article may not have a distinct yet related syndrome.
Asunto(s)
Anodoncia , Cardiomegalia , Enfermedades de la Columna Vertebral , Diente Impactado , Anomalías Múltiples , Anodoncia/patología , Cardiomegalia/patología , Niño , Dedos/anomalías , Trastornos del Crecimiento/patología , Humanos , Discapacidad Intelectual , Masculino , Maloclusión/patología , Mandíbula/anomalías , Micrognatismo/patología , Enfermedades de la Columna Vertebral/patología , Síndrome , Dedos del Pie/anomalías , Anomalías Dentarias/patología , Diente Impactado/patologíaAsunto(s)
Síndrome de Gardner/diagnóstico , Neoplasias Maxilomandibulares/diagnóstico por imagen , Neoplasias Primarias Múltiples/diagnóstico por imagen , Osteoma/diagnóstico por imagen , Femenino , Humanos , Enfermedades Maxilomandibulares/diagnóstico por imagen , Persona de Mediana Edad , Osteítis/diagnóstico por imagen , RadiografíaRESUMEN
Perirenal bleeding following biopsy was assessed in 25 consecutive cases using computerized axial tomography (CT) scanning. Perirenal hematomas were found in 15 patients (60%). In 8 patients the hematomas were moderate or large in size. There was no correlation between the clinical findings, fall in hemoglobin, presence of macroscopic hematuria and the finding of perirenal hematoma by CT scanning.