[Cystic adenomatoid malformation of the lung: experience in 4 operated cases]. / La malformazione adenomatoide cistica del polmone: esperienza in 4 casi operati.

The congenital cystic adenomatoid malformation (MACP), represents the congenital lung malformation most frequent in pediatric age. After examining the embryology, the treatment and the evolution of the malformation we wish to report our experience of four cases of MACP in patients 2 month, 1, 2 and 3 years of age. Lobectomy has cured all patients with the exception of one; this child had a MACP of the lung associated with Rhabdomyosarcoma and died after one and half year of chemotherapy.

Richards-Rundle syndrome, cochleovestibular dysfunction and neurofibromatosis in a family.

The Richards-Rundle syndrome (RRS) is characterized by hearing loss, mental deterioration, ataxia, primary hypogonadism and autosomal recessive transmission. In a sibship of six members we found two sisters with RRS together with baldness, impaired GH and PRL secretion after stimulation and different degrees of impaired insulin secretion. Cochleovestibular investigation of the sibship revealed in each subject more or less severe forms of bulbo-pontine cochleovestibular dysfunction. Three members of the same sibship had cutaneous signs of abortive forms of neurofibromatosis: the son of one of these subjects had a severe form of fully developed neurofibromatosis. Whether there is a pathogenetic linkage between the hereditary multisystemic degeneration (RRS), the dysembryopathy (neurofibromatosis) and the cochleovestibular dysfunction in this family is still not clear.