Paradoxical response to carbonic anhydrase inhibitors in patients with intraretinal cystoid spaces.

Background: Intraretinal cystoid spaces (IRCS) are fluid-filled spaces seen in some retinal dystrophies and often treated with carbonic anhydrase inhibitors. The purpose of this study is to report an unexpected bilateral improvement in the IRCS after discontinuation of therapy. Material and Methods: We identified from our records 23 patients with retinal dystrophy and IRCS who had been treated with topical and/or oral carbonic anhydrase inhibitors. All subjects had regular follow-up with OCT and previous genetic testing. Results: We identified four (17%) patients who experienced a bilateral and symmetrical paradoxical improvement in IRCS size and visual acuity after discontinuation of carbonic anhydrase inhibitors. Two were mutations in RS1, one in CLN3 and another in NR2E3. All patients were followed for at least three years (range 39-63 months). None had systemic abnormalities. Conclusions: Patients with IRCS may exhibit a paradoxical response after discontinuation of carbonic anhydrase inhibitors. Although the pathophysiology of these phenomena is unclear, stopping treatment may be an option in patients who cease to improve or get worse on treatment.

[Prolonged prevention of venous thromboembolic complications (VTECS): who is it indicated for and how should it be carried out?].

The publication contains a review of present-day positions on prolonged prevention of venous thromboembolic complications. This is followed by considering the role of risk factors, probabilistic value of prognostic scales, as well as reflecting the provisions of the recent Russian and international consensus documents concerned. Careful consideration is given to the major possible parameters a physician could be guided by in real clinical practice while choosing and deciding upon appropriate terms of prevention. Also shown are possibilities of using novel oral anticoagulants in practical implementation of such regimens.

[Balanced postoperative analgesia in abdominal surgery: efficiency of the combined use of epidural block and non-opioid analgesics].

One hundred patients who had undergone elective surgery for abdominal malignancy were enrolled in the randomized, controlled study. Postoperative analgesia included only continuous epidural analgesia (PEA) or PEA with intramuscular ketorolac, or PEA with intramuscular ketorolac and intravenous paracetamol. The systemic use of ketorolac and paracetamol in addition to continuous epidural anesthesia can reduce a need for a local anesthetic and the intensity of postoperative movement pain.

[Role of paracetamol in a balanced postoperative analgesia scheme after thoracotomy].

The efficiency of paracetamol used in the balanced multimodal analgesia after thoracotomy still remains unclear. The prospective study covered 75 adult patients operated on the chest. The patients were randomized to 3 groups. They all received epidural autoanalgesia with a mixture of fentanyl (2 microg/ml) in 0.2% ropivacaine solution and intramuscular injections of ketorolac, 30 mg, every 8 hours. In Group 1 (n = 25), paracetamol was intravenously injected in a dose of 4 g daily. In Group 2 (n = 23), rectal paracetamol was used in an equipotential dose. In Group 3 (n = 24), paracetamol was not given. Within the first 24 postoperative hours, the severity of the pain syndrome and the incidence of adverse reactions of analgesia were estimated in all the patients. The obtained data were compared using Student's t-test and x2 test, by taking into account Bonferroni's correction. The p values of < 0.0017 were determined as statistically significant. The resting VAS did not differ between the groups. In cough, the severity of the pain syndrome was significantly less in Group 1 than in Groups 2 and 3. The use of the epidural mixture of ropivacaine and fentanyl required for adequate analgesia within the first 24 hours after surgery was much less in Groups 1 and 2 than that in Group 3. The high incidence of skin itch (20%) and urinary retention (8%) was observed in Group 3 (p < 0.017). No difference was found between the groups in the development of dyspepsia. The use of paracetamol in the postoperative multimodal analgesic therapy program after thoracotomy reduces the daily dose of epidurally administered ropivacaine and fentanyl with evident upgrade of analgesia quality, and the incidence of opioid-induced adverse reactions.

Chorioretinal dysplasia, hydranencephaly, and intracranial calcifications: pseudo-TORCH or a new syndrome?

PURPOSE: To report the association of severe chorioretinal dysplasia, hydranencephaly, microcephaly, and intracranial calcification in children with no evidence of intrauterine infections. METHODS: Two unrelated female infants with visually inattentive behaviour, hydranencephaly, and intracranial calcification were referred for an ophthalmological opinion. RESULTS: The fundus examination and computerised tomograms (CT scans) of head were similar in both children. There was bilateral extensive chorioretinal dysplasia, intracranial calcifications, and hydranencephaly. Serology was negative for acquired intrauterine congenital infections. CONCLUSIONS: We report two cases that may represent a new syndrome or the more severe end of the spectrum of the pseudo-TORCH (toxoplasma, rubella, cytomegalovirus, and herpes simplex) syndrome. The association of chorioretinal dysplasia with the pseudo-TORCH syndrome has not been reported previously.

[Efficiency of preemptive intravenous paracetamol analgesia in abdominal surgery].

In a randomized, controlled study, 50 patients underwent elective surgery for abdominal cancer lesions under perioperative epidural analgesia. All the patients were randomized to receive paracetamol in a single intravenous dose of 1 g or placebo 30 minutes prior to the start of surgery. The use of 1 g of paracetamol as a single intravenous preemptive dose in abdominal surgery with perioperative epidural analgesia does not reduce the consumption of the analgesic and the intensity of pain in the postoperative period.

[Use of valvular bronchoblocation in the complex treatment of patients with disseminated pulmonary tuberculosis complicated by pulmonary hemorrhage].

The results of treatment are analyzed in 102 patients with disseminated pulmonary tuberculosis complicated by pulmonary hemorrhage. The authors have developed and clinically tested a procedure for arresting pulmonary hemorrhage by creating therapeutic hypoventilation and atelectasis of a lung portion, in which there is a source of bleeding, by using valvular bronchoblocation of the draining bronchus. The application of valvular bronchoblocation to patients with pulmonary bleeding enhances the efficiency of complex treatment and reduces mortality by 4.9 times and a need for emergency and urgent surgical interventions by 7.4 times.

[Use of endobronchial valve in postresection empyema and residual cavities with bronchopleural fistulas].

The results of treatment are analyzed in 78 patients with brochopleural fistilas after lung surgery. A method for imaging the draining bronchus under endoscopic guidance, by using the foamed dye administered into the residual cavity, has been developed. A valvular bronchial blocker for abolishing the function of bronchopleural fistular inserted into the lobular and segmental bronchus at bronchoscopy was designed and clinically tested. This procedure allows resurgery to be avoided in 91.7% of cases.

Risk of new-onset uveitis in patients with juvenile idiopathic arthritis treated with anti-TNFalpha agents.

OBJECTIVE: To determine whether treatment with tumor necrosis factor alpha (TNFalpha)-blocking agents alters the incidence of new-onset uveitis in patients with juvenile idiopathic arthritis (JIA). STUDY DESIGN: Cohort study based on retrospective chart review. The charts of all 1109 patients with a diagnosis of JIA seen between January 1, 1996, and June 30, 2003, at our clinic were reviewed for diagnosis of uveitis and treatment with TNFalpha inhibitors. Cox regression analysis was performed with anti-TNFalpha treatment as a time-dependent covariate for risk of development of uveitis. RESULTS: We identified 70 patients treated with anti-TNFalpha without a prior diagnosis of uveitis. Two of these 70 patients (2.9%), both treated with etanercept, had development of new-onset uveitis during anti-TNFalpha therapy. One had juvenile psoriatic arthritis diagnosed 4.1 years before onset of uveitis. The other had extended oligoarticular JIA diagnosed 6.4 years before onset of uveitis. We found no statistically significant difference in the risk for development of uveitis between patients with or without anti-TNFalpha treatment. CONCLUSIONS: In our patients with JIA, anti-TNFalpha treatment did not alter the risk for development of new-onset uveitis. However, anti-TNFalpha therapy with etanercept did not prevent the development of uveitis in 2 patients.

Tumour necrosis factor alpha inhibitors in the treatment of childhood uveitis.

OBJECTIVE: To describe the efficacy of anti-TNF-alpha agents in the treatment of childhood uveitis. METHODS: We performed a retrospective chart review of all children with uveitis treated with TNF-alpha blockers at The Hospital for Sick Children, Toronto. RESULTS: Twenty-one children with uveitis were treated with the anti-TNF-alpha agents etanercept (11 patients) and infliximab (13 patients), resulting in 24 treatment courses. All patients had persistently active uveitis despite treatment with at least one standard immunosuppressive drug before the start of anti-TNF-alpha therapy. Six of 21 patients (29%) had idiopathic uveitis. In the other 15 patients, the underlying disease was juvenile idiopathic arthritis in 12 (57%), Behçet disease in two (9%) and sarcoidosis in one (5%). Response to etanercept treatment was good in 27%, moderate in 27% and poor in 45% of patients. Response to infliximab treatment was good in 38%, moderate in 54% and poor in 8% of patients. The difference in the percentage of patients with a moderate or good response was statistically significant (P = 0.0481). We also observed a lower rate of complications, such as new-onset or worsening glaucoma or cataract in the infliximab-treated group. CONCLUSION: Anti-TNF-alpha treatment was beneficial in a high percentage of patients with childhood uveitis refractory to standard immunosuppressive treatment. Infliximab resulted in better clinical responses with less ocular complications than etanercept.

The role of nasal endoscopy in repeat pediatric nasolacrimal duct probings.

PURPOSE: To determine whether pediatric nasal endoscopy improves treatment success in recurrent nasolacrimal duct obstruction. METHODS: A retrospective analysis was carried out on 2 nonrandomized cohorts of consecutively treated patients who had one failed nasolacrimal duct probing at the Hospital for Sick Children, University of Toronto. The first cohort of 24 consecutive patients (32 eyes), group A, was treated with Crawford silicone tube intubation by one ophthalmologist (R.C.P.). The second cohort of 23 consecutive patients (33 eyes), group B, was treated with repeat probing by a second ophthalmologist (A.V.L.) in conjunction with nasal endoscopy by one otolaryngologist (V.F.). Abnormalities found on endoscopy were treated accordingly, and no tubes were inserted. Follow-up, through phone interviews or office visits, was conducted to assess the patients' symptoms. RESULTS: Group A patients were older at both the first probing (P = .048) and the second procedure (P = .012). No significant difference in the failure rates was found, with treatment failing in 3 eyes (2 patients) in group A and in 5 eyes (4 patients) in group B (P = .479). Interestingly, 17 of the 32 tubes in group A were extruded in 1 month or less. Also, 28 of 33 eyes in group B had abnormalities on endoscopy-some, multiple. Twenty-two eyes underwent inferior turbinate infracture: 5 had redundant mucosa, which was removed, and 6 had abnormal openings of the inferior meatus. CONCLUSIONS: We were unable to show any benefit of nasal endoscopy over intubation of the nasolacrimal system with silicone tubes in the treatment of failed probings despite the identification and treatment of abnormalities. The study was limited by its low power to detect differences because of the small number of patients and the high success rate of the traditional treatments for congenital nasolacrimal duct obstruction.

Bioethics for clinicians: 23. Disclosure of medical error.

Adverse events and medical errors are not uncommon. In this article we review the literature on such events and discuss the ethical, legal and practical aspects of whether and how they should be disclosed to patients. Ethics, professional policy and the law, as well as the relevant empirical literature, suggest that timely and candid disclosure should be standard practice. Candour about error may lessen, rather than increase, the medicolegal liability of the health care professionals and may help to alleviate the patient's concerns. Guidelines for disclosure to patients, and their families if necessary, are proposed.

A prospective evaluation of anterior transposition of the inferior oblique muscle, with and without resection, in the treatment of dissociated vertical deviation.

BACKGROUND: Anterior transposition of the inferior oblique muscle (ATIO) has become a popular surgical treatment for dissociated vertical deviation (DVD), particularly in patients with coexisting inferior oblique muscle overaction (IOOA). We wanted to assess whether adding a resection improves the outcome compared with standard anteriorization. METHODS: We undertook a prospective, randomized evaluation of ATIO, with and without a 7-mm resection, in patients with DVD of at least 5 PD in one eye. We included 51 eyes of 30 patients, 26 eyes treated with the standard ATIO and 25 treated with a 7-mm resection added. We recorded the size of the preoperative and final DVD, grade of the preoperative and final IOOA, rates of reoperation, and complications. Mean follow-up was 15.4 months in the standard group and 25.0 months in the resection group, with a minimum of 4 months for all cases. RESULTS: The median preoperative and postoperative DVD was 12 PD and 4 PD in the standard group, respectively. This compared with 14 PD and 4 PD, respectively, in the resection group, representing no statistically significant difference in outcome. The presence or absence of IOOA did not influence the result of ATIO for either group. No significant complications of surgery occurred in either group. CONCLUSIONS: ATIO is an effective treatment for DVD and can be used to treat DVD in patients with or without IOOA, with few adverse effects. Our study revealed no advantage to adding a 7-mm resection to the standard procedure.

Ultrasound biomicroscopy of the eye in cystinosis.

OBJECTIVE: To describe the ocular ultrasound biomicroscopy (UBM) findings in patients with cystinosis. METHODS: Six patients with infantile nephropathic cystinosis, aged 16 to 25 years, and 6 controls (matched for age and spherical refractive error) were examined clinically and with UBM. Scleral reflectivity, corneal and iris thickness, central anterior chamber depth, angle width, trabecular meshwork to ciliary process distance, and ciliary sulcus width were measured. RESULTS: No patient had glaucoma or posterior synechiae, but all had crystals in the trabecular meshwork apparent with gonioscopy. Using UBM, the cornea and iris appeared similar in both groups, but the scleral reflectivity was increased in patients (P =.003). The angle was narrower in patients (mean +/- SD, 20 degrees +/- 7 degrees ) than controls (31 degrees +/- 5 degrees, P<. 001). The anterior chamber was shallower in patients (2556 +/- 197 microm) than controls (2968 +/- 284 microm, P<.001). The ciliary sulcus was closed or narrow in all patients (83 +/- 112 microm) compared with controls (339 +/- 135 microm, P<.001), with a reduction in the trabecular meshwork to ciliary process distance. CONCLUSIONS: This report of ocular UBM findings in cystinosis demonstrated narrowing of the angle and a ciliary body configuration similar to that reported for plateau iris syndrome. Gonioscopy demonstrated crystals in the trabecular meshwork. These findings may explain the predisposition of these patients to glaucoma.

Molecular characterisation of congenital glaucoma in a consanguineous Canadian community: a step towards preventing glaucoma related blindness.

Glaucoma is a leading cause of irreversible blindness in Canada. Congenital glaucoma usually manifests during the first years of life and is characterised by severe visual loss and autosomal recessive inheritance. Two disease loci, on chromosomes 1p36 and 2p21, have been associated with various forms of congenital glaucoma. A branch of a large six generation family from a consanguineous Amish community in south western Ontario was affected with congenital glaucoma and was studied by linkage and mutational analysis to identify the glaucoma related genetic defects. Linkage analysis using the MLINK component of the LINKAGE package (v 5.1) showed evidence of linkage to the 2p21 region (Zmax=3.34, theta=0, D2S1348 and D2S1346). Mutational analysis of the primary candidate gene, CYP1B1, was done by direct cycle sequencing, dideoxy fingerprinting analysis, and fragment analysis. Two different disease causing mutations in exon 3, 1410del13 and 1505G-->A, both segregated with the disease phenotype. The two different combinations of these alleles appeared to result in a variable expressivity of the phenotype. The compound heterozygote appeared to have a milder phenotype when compared to the homozygotes for the 13 bp deletion. The congenital glaucoma phenotype for this large inbred Amish family is the result of mutations in CYP1B1 (2p21). The molecular information derived from this study will be used to help identify carriers of the CYP1B1 mutation in this community and optimise the management of those at risk of developing glaucoma.

The epidemiology of pediatric glaucoma: the Toronto experience.

BACKGROUND: This study was conceived to provide an insight into the spectrum of glaucoma in the pediatric population. We also set out to compare the success of disease control and the prognosis for vision within the different diagnostic subgroups. This is the largest single population of children with glaucoma that has been so described and compared. METHODS: The charts of children who were first seen between birth and age 16 years and who attended the Hospital for Sick Children with any form of glaucoma between January 1974 and January 1995 were reviewed and entered into the study. RESULTS: Data are presented for 306 children. Congenital glaucoma was the most common subtype, accounting for 38%. Patients with congenital glaucoma were young, had surgery, and had more operations than any other group except those with aniridia. Goniotomy offered a cure in 47.8% of the patients. A bimodal distribution reflected their visual performance. Patients with aphakic glaucoma, the next most prevalent group (20%), presented at an older age (4.5 years). Surgical intervention was performed in 50% of these children. Nearly all patients with Sturge-Weber syndrome (80%) had surgery. The following glaucoma groups were associated with a poor visual outcome: aniridia, anterior segment developmental anomalies involving the cornea, uveitis with glaucoma other than steroid induced, retinopathy of prematurity, and persistent hyperplastic primary vitreous. Steroid-induced glaucoma and anterior segment dysgenesis, excluding Peters anomaly, had uniformly good outcomes. CONCLUSION: The ability to control glaucoma in childhood and visual prognosis is highly variable. Particular diagnostic categories do consistently well and some do poorly.

Tubulointerstitial nephritis and uveitis in children and adolescents. Four new cases and a review of the literature.

We identified 35 cases of tubulointerstitial nephritis and uveitis (TINU), 31 from a MEDLINE search (1966-1996) of the English literature and 4 from our hospital records (1988-1996). To meet the case definition, the patient had to be less than 18 years old and have TINU of unknown cause. Common presenting symptoms included fatigue, weight loss, fever, and abdominal pain. The uveitis was usually anterior and could occur at any time with respect to the onset of the renal disease. Common laboratory features included anemia, increased erythrocyte sedimentation rate, and decreased creatinine clearance. Most patients (33 of 35) had renal biopsies that commonly revealed an intense inflammatory interstitial infiltrate, glomerular sparing, and negative immunofluorescence studies. Of the 35 patients, 26 received systemic corticosteroid therapy (5 of 26 for eye disease); 22 had follow-up for at least 1 year; 13 of 35 patients had a recurrence of their uveitis. The outcome in all 35 cases was normal renal function with no documented visual loss. In conclusion, TINU is a unique syndrome with characteristic clinical features, laboratory changes, and renal biopsy results. Treatment is controversial, and the outcome in children, even if untreated, is excellent.