Childhood fibrous dysplasia presenting as blindness: a skull base approach for resection and immediate reconstruction

Fibrous dysplasia is an abnormal fibroosseous process of bone of unknown cause. The incidence of skull involvement varies, painless enlargement being the most common presenting symptom. Change in vision is a rare but recognized finding. We report a 3-year-old boy with extreme fibrous dysplasia involving the skull base, who presented with blindness. He underwent exposure osteotomies of the frontal bones and orbits to provide access for skull base tumor removal. The orbital roofs were reconstructed with microplate-fixed cranial grafts. One and one half years after tumor excision followed by immediate reconstruction, the boy retains facial symmetry, and his ocular function has not deteriorated.     

Treatment and prognosis of secondary glaucoma in Fuchs' heterochromic iridocyclitis

After reviewing the records of 111 patients with Fuchs' heterochromic iridocyclitis, we studied the therapy and prognosis of secondary glaucoma in 30 of these 111 patients (27%) who had glaucoma or could be considered glaucoma suspects. Maximal medical therapy was unsuccessful in 22 of the 30 patients (73%). Surgical intervention (mostly trabeculectomies, half with 5-fluorouracil) successfully controlled intraocular pressure (< or = 21 mm Hg with or without medication) in 13 of the 18 operated-on patients (72%) after a mean follow-up of 26 months. All successfully operated-on patients retained a visual acuity of 20/80 or better. We had favorable results, possibly because of modern surgical techniques (use of 5-fluorouracil, sodium hyaluronate) or earlier surgical intervention, or both.     

STRONG METAL-CERAMIC JOINTS

In this paper the technology for joining metals to ceramics is reviewed. The technologies of metal powder sintering, liquid phase joining with activated brazing alloys and with ceramic frit, solid-state joining, friction welding and mechanical fixation are described. In order to put some perspective on the mechanical data given in the literature, some evaluating remarks on mechanical testing are made.     

Intubation conditions: importance of the rate of repetition of the train-of-four

OBJECTIVES: To assess that neuromuscular relaxation onset of the adductor pollicis (AP) is related to neuromuscular stimulation rate. To assess that train-of-four (TOF) at 0.05 Hz is a more accurate indicator of optimal tracheal intubation time and conditions, than TOF at 0.08 Hz. STUDY DESIGN: Prospective, comparative, randomized double-blind study. PATIENTS: Forty adults, physical class ASA 1 or 2, undergoing general anaesthesia with tracheal intubation were allocated to two groups (n = 20) according to the sequence of stimulation of the AP: either TOF at 0.05 Hz (test group) or TOF at 0.08 Hz (control group). METHODS: Induction of anaesthesia was achieved with thiopentone, fentanyl and vecuronium (0.1 mg.kg-1). Neuromuscular monitoring was obtained with force displacement transducers attached to each AP. Tracheal intubation was performed once AP muscular response obtained with TOF at 0.05 Hz for test group and TOF at 0.08 Hz for control group was abolished. Results are expressed as mean +/- SEM. Fisher exact test was used for intubation conditions comparison. Curarization time between groups was compared with unpaired Student's t test (P < 0.05 accepted). RESULTS: TOF with 0.05 Hz stimulation significantly increased curarization time: 217 +/- 7 versus 162 +/- 6 s (P < 0.001). Intubation conditions were excellent in 95% and good in 5% of patients in the study group, compared to 15 and 40% in the control group, respectively (P < 0.01) in 45% of the control group patients coughing at intubation occurred. CONCLUSION: Low stimulation rate (TOF at 0.05 Hz) of AP is a reliable technique to determine the appropriate intubation time for patients paralyzed with vecuronium.     

trans-2-phenylcyclopropylamine is a substrate for and inactivator of horseradish peroxidase

Horseradish peroxidase (HRP) is well known for mediating the electron-transfer oxidation of electron-rich aromatic 'donors' such as phenols and anilines, but has not been described to oxidize aliphatic amines. We here confirm the inability of HRP to oxidize typical aliphatic amines, even those which would exist significantly as free bases at the operative pH. In contrast, trans-2-phenylcyclopropylamine (2-PCPA) is both a substrate (turnover product is cinnamaldehyde) and a time-dependent inactivator of HRP. These activities of 2-PCPA are consistent with either a concerted or rapid sequential one-electron-oxidation/ring-opening to give an intermediate capable of covalent binding to the enzyme. 2-PCPA is the first known example of a simple aliphatic amine which serves as a substrate for HRP under turnover conditions.     

Diagnosis of systemic causes of uveitis; a matter of ophthalmologist and internist

OBJECTIVE: To assess how often the aetiology is established in patients with uveitis, what systemic disease are found and what is the contribution of the internist to the diagnostic process. DESIGN: Retrospective study. SETTING: University Hospital Leiden, the Netherlands. METHOD: From January 1987 to April 1992, 342 patients presented with uveitis. All patients underwent a standard ophthalmological examination. Referral to an internist and individualised laboratory screening followed in patients with recurrent, chronic, bilateral or panuveitis. Recorded were: ophthalmological data, results of laboratory screening, results of analysis by the internist, final diagnosis and presence of systemic disease. RESULTS: 149 (44%) patients were examined by the internist, 18 (5.2%) were seen by another specialist. In 169 (49%) patients a specific diagnosis was made. 74 (22%) had a systemic disease, 74 a primary ocular disease. In 28 (8%) a systemic disease was presumed (5% were HLA-B27 positive, 3% had abnormal laboratory results); 5 (1%) patients had endophthalmitis as a complication of a septic process. CONCLUSION: In approximately 1/3 of the patients with uveitis a systemic disease was found. Examination by the internist tailored to the individual patient is essential in the evaluation of uveitis patients.     

Behavior of a vibrating wire near the liquid3He-vacuum interface and in a saturated3He-4He mixture

In this paper we describe the effect of the vicinity of a liquid³He-vacuum interface on the behavior of a vibrating wire viscometer. It was found that in the fluid near the liquid interface the quality factor is lower than in the bulk liquid. We further report on the observation of a doubling of the resonance peak of the wire in a saturated³He-⁴He mixture. The frequencies and amplitudes of the two peaks strongly depend on the distance between the phase boundary and the vibrating wire; the temperature and the velocity of the phase boundary have no significant influence on the peak frequencies. The observed peak doubling is attributed to the coupling of the vibrating wire with a standing second-sound wave in the dilute phase where the volume of the dilute phase can be regarded as a resonating cavity for second sound.