Hemifacial microsomia: use of the OMENS-Plus classification at the Royal Children's Hospital of Melbourne

Hemifacial microsomia is the most common facial congenital disability after cleft lip and palate, but as yet its pathogenesis remains unknown. Clinical classification systems have evolved over the last 30 years from those classifying only single components of the disorder, to those classifying according to the combination of deformities, to the most recent systems that grade each anatomical component separately, such as the Orbit, Mandible, Ear, Nerve, and Soft tissue (OMENS) system. The aim of the present study was to review the classification of patients with hemifacial microsomia treated by the Melbourne Craniofacial Unit at the Royal Children's Hospital using the OMENS-Plus system of classification and to correlate the findings with data from other centers. Records of patients treated by the craniofacial unit were reviewed and included in the study if adequate clinical records, photographs, and radiographs (anteroposterior, lateral, basal cephalometry, panoramic views) were available. The data were entered into a database file developed for this purpose. Seventy-one patients were identified from the hospital database, of which six were excluded because of incomplete data. Of the 65 patients, there were 31 (48 percent) with right-sided microsomia, 25 (38 percent) with left-sided microsomia, and nine (14 percent) with bilateral microsomia, with an overall male-to-female ratio of 1.2:1. The majority of patients had a normal orbit (77 percent), mildly hypoplastic mandibular ramus-condyle with functioning temporomandibular joint (57 percent with type M1 or M2a), normal facial nerve (76 percent), and mild soft-tissue hypoplasia (73 percent). There was a similar proportion of patients with mild ear anomalies (53 percent with grade 0 or 1) compared with those with more severe anomalies (47 percent with grade 2 or 3). Correlative analysis demonstrated a slight but positive correlation between the severity gradings of the five individual components. The correlation was lowest between the grading of the nerve and ear and that of the mandible and nerve. The data demonstrate the phenotypic variability of hemifacial microsomia and suggest a degree of relationship among the components of hemifacial microsomia. The OMENS-Plus system has provided a major advancement in the classification of hemifacial microsomia. The authors suggest refinements to the grading of the orbit and nerve components.     

The Hyperbaric Chamber at the Royal Victoria Hospital,Montreal

The single apparent and potential benefit of hyperbaric oxygen is the great increase in the blood content of dissolved oxygen achieved when pure oxygen is breathed at increased pressure. The design of an economical chamber for this purpose is presented. A large number of physiological measurements (cardiac output, electroencephalogram, electrocardiogram, etc.) can be performed on patients or experimental animals within the chamber by use of unique electronic connections in the chamber wall which permits all recording equipment to remain outside. Expected arterial blood oxygen tensions have been achieved in patients studied at 2, 3, and 4 atmospheres. Safety features are emphasized. No complication has resulted in 113 dives over the period January to June 1964, one-half of which were for treatment of patients. The chamber has been used clinically as an adjunct to treatment of shock, certain forms of malignancy, anaerobic infections, coronary occlusion, and problems of ischemia, and for preservation of organs for transplantation.     

ΔF508 testing of the DNA bank of the Royal Manchester Children's Hospital

Summary Details of haplotype and ΔF508 status from various populations represented in the cystic fibrosis (CF) DNA bank of the Royal Manchester Children's Hospital are provided, together with information on the association of genotype and clinical status. Clinical details and DNA analyses from native English in the North-West and South-West of England (Bath), from Lancashire Pakistani families and from Afrikaans Namibian families are compared. A 78.5% incidence of ΔF508 has been found in English families. Compound heterozygotes with CF and only one ΔF508 gene have an increased likelihood of having milder disease, with lessPseudomonas isolated from sputum and relatively more showing either no regular respiratory pathogens or colonisation withStaphylococcus. There is also a relative increase in meconium ileus in these compound heterozygotes. The diagnosis of CF may be in doubt in some subjects negative for ΔF508. Some of the Bath families have unusual haplotypes for an English population and a compound heterozygote ΔF508/ΔI507 has been found. There is evidence from metD analysis of the founder effect in the Afrikaans Namibian families, who have a high ΔF508 incidence.