RESUMEN
Poland’s syndrome is a rare congenital anomaly consisting of a unilateral absence of the pectoralis major, ipsilateral muscle, hand anomaly and occasionally associated other malformations of the chest wall and breast. Many structural and functional abnormalities have been described in association with this syndrome. We report an incidentally diagnosed case in a 27-year-old male patient who presented to us with symbrachydactyly. In addition to this, anterior depression of 2nd, 3rd and 4th ribs and bifid (forked) 5th rib was present on radiological investigations. The body of sternum was short and deformed on the right side with absence of xiphoid process. All middle phalanges were absent on right hand. It is a rare variant of Poland’s syndrome.
Asunto(s)
Adulto , Dedos/anomalías , Dedos/diagnóstico por imagen , Humanos , Masculino , Enfermedades Musculares/complicaciones , Músculos Pectorales/anomalías , Síndrome de Poland/complicaciones , Costillas/anomalías , Sindactilia/complicacionesAsunto(s)
Tobillo/anomalías , Estatura , Huesos/anomalías , Niño , Femenino , Hipoplasia Dérmica Focal/complicaciones , Trastornos del Crecimiento/complicaciones , Humanos , Deformidades Congénitas de las Extremidades/complicaciones , Escoliosis/complicaciones , Sindactilia/complicaciones , Dedos del Pie/anomalías , Anomalías Dentarias/complicacionesRESUMEN
A 53 year-old woman presented a recurrent bifrontal headache of 2 years duration and bilateral progressive visual disturbance. The clinical and neurological examination showed a bilateral feet adactyly and bitemporal hemianopsia. The brain MRI demonstrated a Rathke's cleft cyst. The patient was operated by a transnasal endoscopic approach. It seems that this unusual association has never been described before.
Mulher de 53 anos com história recorrente de cefaléia com duração de 2 anos bilateral e progressiva, acompanhada de distúrbios visuais. O exame clinico e neurológico mostrou uma adactilia dos pés e hemianopsia bitemporal. A ressonância nuclear magnética cerebral mostrou um cisto de Rathke. A paciente foi operada por via transnasal endoscópica. Aparentemente esta é a primeira vez que esta associação é descrita na literatura.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Quistes del Sistema Nervioso Central/complicaciones , Neoplasias Hipofisarias/complicaciones , Sindactilia/complicaciones , Dedos del Pie/anomalías , Quistes del Sistema Nervioso Central/diagnóstico , Quistes del Sistema Nervioso Central/cirugía , Imagen por Resonancia Magnética , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/cirugíaRESUMEN
Lobster Claw Syndrome is a type of syndactyly, where abnormalities in the hand-foot region as well as the orodental region occur. A case of a 14-year old boy with this syndrome is presented here.
Asunto(s)
Adolescente , Displasia Ectodérmica/complicaciones , Humanos , Anomalías Maxilomandibulares/etiología , Masculino , Sindactilia/complicaciones , Síndrome , Anomalías Dentarias/etiologíaRESUMEN
Os autores apresentam dois pacientes portadores de epidermólise bolhosa, com lesoes generalizadas, sindactilias nas maos, deformidade em flexao dos dedos e aduçao do polegar. Discutem a patologia, as peculiaridades do tratamento nas maos e relatam seus resultados.