The Wyburn-Mason syndrome (author's transl)

A patient aged 39 years with Wyburn-Mason syndrome is presented. He had an apple-sized intracranial arteriovenous angioma racemosum of the left temporo-medical region, which was inoperable, as it extended to the basal ganglia. It was diagnosed by carotid angiography and by sequential brain szintigraphy. More than 50 retinal arteriovenous anastomoses were observed in the left eye, involving all quadrants and the macular region. Some of these anastomoses were only found by fluorescence angiography. Clinical signs were hemiparesis and decreased sensibility of the right side, headaches, speech disorders and right homonymous hemianopia. Vision was decreased to 0.4. By partial embolisation via the right common carotid, it was possible to reduce the size of the intracranial hemangioma.     

Osteodysplasty (Melnick-Needles syndrome) (author's transl)

Authors report a new case of osteodisplasty (Melnick-Needles syndrome) and describe radiological findings that are typical of this entity: bony sclerosis of base structures, abnormal dental alignment, sclerosis and irregularity of clavicles and ribs, described as "band-like" deformity, cortical irregularity of tubular bones with metaphyseal flaring and diaphyseal bowing. Flat bones are broadened, particularly iliac crest and vertebral bones. Facial features described by the majority of authors in this entity are present in our patient in an incomplete form: craniofacial disproportion, micrognatia, depressed temples, exophthalmus (not in our patient) and broadened forehead. The paper is intended to gather clinical data of already reported cases in order to facilitate clinical research in the future from the immunological, genetical and biochemical points of view. Our patient has the same clinical history as the patient studied in the original paper. Dr. Melnick was kind enough to send us clinical data and autopsy findings.     

Vater or Vacterl syndrome (author's transl)

Analysis of malformations in 65 newborns with limb anomalies, 39 with esophageal atresia with tracheoesophageal fistula, and 41 with anal atresia confirmed the nonrandom tendency for the defects of the VATER or VACTERL syndrome to associate together. 11 new patients with 4 or more of these anomalies were compared with 41 previously reported cases. There was good agreement with reference to the frequency of the major malformations noted in the VACTERL association. While anal atresia was not so common in our patients, cardiac anomalies and radial limb dysplasia occurred somewhat more frequently. In accordance with previous findings we also emphasize a single umbilical artery as one of the malformations in the spectrum of the VACTERL association (V = vertebral defects and vascular anomalies). Because of the high incidence of rib anomalies in our patients and in earlier cases with complete medical records it is suggested that the scope of the VACTERL association should be enlarged by this malformation. Thus the R in VACTERL would stand not only for renal defects but als for rib anomalies. Furthermore, the spectrum of anomalies could be extended by auricular defects (A = anal atresia and auricular defects). When one of these VACTERL components is found attention should be drawn to the possibility of the presence of the other associated anomalies. The developmentally correlated malformations seen in the VACTERL syndrome are generally sporadically observed. At the present time the etiology is unknown but heterogeneity is suggested. Although a causal relationship between maternal intake of progesteron/estrogen during the vulnerable period of embryogenesis and the VACTERL syndrome has been suggested, none of the mothers of our patients were exposed to these hormones during early pregnancy. Cytogenetic investigation in one patient and his mother showed a so-called marker chromosome 9 (C9qh+ variant) which is difficult to interpret at the present time.     

Two cases of external ophthalmomyasis (author's transl)

After a brief survey of Hungarian references the author reports on two cases of ophthalmomyasis externa. The organism was shown to be present oestrus ovis in one instance. The treatment with carbon-tetrachloride might be considered as a new and efficacious therapy.     

Adhesive otitis: 30 surgical cases (author's transl)

Adhesive otitis accounts for 3% of all cases of chronic otitis. It is characterized by the attachment of the entire epidermal layer of the drum to the inner wall of the cavity. Three causative factors are identified: abnormal permeability of the auditory tube; absence of the fibrous layer of the tympanic membrane; a predisposing milieu. This is a very different condition from tympanosclerosis and its scleroadhesive sequelae. It can, however, be associated with cholesteatoma. An analysis of 30 cases in which surgery was performed points to the real progress brought about by the use of tympanic homografts. One problem remains: permanent aeration of the new tympanic cavity.