Cleidocranial dysostosis is a rare genetic syndrome with an autosomal dominant inheritance pattern. The most common manifestations include clavicular aplasia or hypoplasia, open fontanelles and abnormal dentition. The present report describes two familial cases whose late diagnosis was made by means of clinical and radiographic findings. The treatment was radical, with complete surgical teeth extraction and making of total dental prosthesis.

Apresenta-se o caso de uma paciente com nefrolitíase que, após submeter-se a uma litotripsia, evoluiu com pielonefrite xantogranulomatosa, fístula nefrobrônquica, abscesso pulmonar e septicemia. O tratamento incluiu drenagem da loja renal, drenagem tubular da pleura e do abscesso pulmonar por toracotomia e nefrectomia direita. A bactéria isolada, tanto da loja renal, quanto do abscesso pulmonar, foi a Pasteurella aerogenes, sendo este o primeiro caso na literatura médica mundial relacionado a este patógeno.

The authors report the case of a 58 year-old woman with nephrolithiasis, who after being submitted to percutaneous lithotripsy developed xantogranulomatous pyelonephritis, lung abscess and sepsis. Initially, treatment included open drainage of the retroperitoneum, nephrectomy and tubular drainage of the lung abscess and pleural space, through right lateral thoracotomy. Pastereula aerogenes was isolated from both lung and retroperitoneal secretions. To our knowledge, this is the first case of nephrobronchial fistula related with this bacteria in medical literature.