A 4 4/12‐year‐old boy with the Poland‐Moebius syndrome, whose mother had the Poland syndrome, is presented. This is the first report of the occurrence of both syndromes in the same family, suggesting that they are expressions of the same autosomal dominant gene.

Describir sobre la base de disecciones de piezas anatómicas, los componentes anatómicos del Complejo Articular Cràneo-Mandibular (CACM). Materiales y métodos: Se utilizaron 08 hemicabezas de preparados anatómicos pertenecientes a adultos humanos venezolanos, tratados previamente con formol al 10% y conservados en Gerdex ® sin diluir durante 6 meses, previo a la práctica manual. Consecutivamente, se disecaron los elementos siguiendo el protocolo de la Facultad de Odontología de la Universidad de los Andes y se particularizaron las piezas anatómicas con la finalidad de abordar la diferente naturaleza de los elementos que conforman el CACM. Resultados: La disección anatómica logró contrastar lo referido desde el punto de vista teórico con relación a lo que puede evidenciarse en una intervención quirúrgica, ilustrando el contenido anatómico que la misma posee en condiciones de normalidad. Los elementos del CACM en su mayoría coinciden notablemente con lo descrito en esta investigación; por lo tanto, el conocimiento concreto de estos componentes permite una preparación previa para evitar cualquier complicación o alteración de las condiciones anatómicas de estas estructuras. Conclusiones: Al aplicar esta técnica se contribuyó en el campo investigativo a ampliar el proceso de aprendizaje anatómico teórico-práctico para los profesionales de la Odontología.

All three sibs from normal and unrelated parents had congenital ectopia lentis without extraocular abnormalities. This pedigree suggests an autosomal recessive form of simple ectopia lentis.The aim of this report is to describe three sibs affected with ectopia lentis without corectopia, suggesting an autosomal recessive form of simple ectopia lentis. CASE REPORTSCase A. The three-year-old propositus was the product of an uncomplicated eight-month twin pregnancy and delivery by cesarean section. Birthweight was 2500 g. Tremulous irides have been present since birth. His psychomotor development and somatometric measurements (weight, W : 17.9 kg, height, H : 98 cm, arm span, AS: 98 cm, lower segment, LS: 43 cm, and cephalic circumference, CC: 49 cm) were within normal limits. The hair was blond and the eyes green. Ophthalmological examination revealed central fixation in the right eye and eccentric in the left. Normal corneas, central pupils, and wide anterior chambers were found. Irides were tremulous and showed remnants of the pupillary membrane con-

A 37-year-old male had clinical and electrophysiological features of hereditary motor and sensory neuropathy (neuronal type) with onset in infmcy, as well as histological picture of neurogenic myopathy. Two sons, aged 2 and 3 4/12 years, showed congenital contraction deformities of feet. delayed motor development, and electrophysiological features similar to those of the father. All three also presented laryngeal abnormalities, peculiar facies, short neck, narrow shoulders and protruding chest. The authors conclude that this aggregate of anomalies constitutes a "new" syndrome probably due to an autosomal dominant gene.

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