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Control of hydrocephalus by endoscopic choroid plexus coagulation--long-term results and complications.

Endoscopic choroid plexus coagulation has been used for the control of hydrocephalus regularly at this unit for the past 20 years. 156 of these operations have been performed on 116 patients and the aim of this study was to assess the rate of long-term control of hydrocephalus and the complications of the procedure. Data have so far been found for 98 patients with a median age at operation of 5 months (range 1 week-30 years). After a mean follow-up period of 10.5 years there were 32 (33%) patients continuing without a ventricular shunt. One patient developed papilloedema and required ventricular shunting 16 years after choroid plexus coagulation. There were no deaths resulting from operation. 5 patients developed post-operative meningitis and 3 patients had post-operative infections of implants (2 shunts and 1 reservoir). No cases of post-operative meningitis have occurred since vancomycin and gentamicin have been added to the solution used to perfuse the ventricles after operation (28 cases). Other complications included post-operative fits (2), respiratory arrest in a premature baby (10, low-pressure state (1), blocked or leaking external ventricular drain (4), drain displacement (4), subdural effusion (1) and per-operative minor ventricular bleeding forcing abandonment of the procedure (2).

Adolescent↗

Measurement of scleral thickness in uveal effusion syndrome.

PURPOSE: To measure scleral thickness in patients with and without uveal effusion syndrome using ultrasound biomicroscopy (UBM) and magnetic resonance imaging (MRI). DESIGN: Prospective case-control study. METHODS: UBM was used to measure scleral thickness in five subjects with uveal effusion syndrome and five matched controls. We also used MRI to measure scleral thickness in three subjects. RESULTS: The mean thicknesses for eyes with uveal effusion syndrome versus control eyes were 0.65 +/- 0.08 mm and 0.55 +/- 0.05 mm, respectively (mean difference 0.10, P value = .13). MRI measurements of three subjects showed abnormally thick sclera but were imprecise. CONCLUSIONS: UBM can be used to measure scleral thickness, and our results support the finding that patients with uveal effusion syndrome have abnormally thick sclera. Compared with MRI, UBM may be a more accurate and precise method of measuring scleral thickness. UBM can be a useful adjunctive test in the management of uveal effusion syndrome.

Aged↗

Posterior microphthalmos.

Posterior microphthalmos is a congenital anomaly of the posterior segment of the eye, caused by an abnormally thickened sclera. The thickened sclera does not impede the growth of the neuro-retina but seems to influence the development of the choroid and of the retinal pigment epithelium. This may explain the common occurrence of a papillomacular fold in such cases. As such eyes are at risk of developing uveal effusion or angle-closure glaucoma, it is important to consider the diagnosis of posterior microphthalmos in high hypermetropic eyes.

Adult↗

Lateral ventricle choroid plexus papilloma in childhood: management and complications.

BACKGROUND: A review of the choroid plexus papilloma of the lateral ventricle in the pediatric age group is presented. The characteristic clinical features, imaging studies, preoperative, and operative approaches, as well as complications, will be included. METHODS: Among a group of 24 patients with ventricular choroid plexus papilloma treated by the authors over a 12-year period, seven patients were selected for this study. The selection included patients under the age of 8 years at the time of their presentation with choroid plexus papilloma of the lateral ventricle. Adult patients and children with choroid plexus papilloma at other sites or choroid plexus carcinoma were excluded. At least a 30-month follow-up period was available for all patients except one. Preoperative and postoperative imaging studies confirming total tumor resection were available for all patients. RESULTS: Developmental delays and regression were the most consistent clinical features. Magnetic resonance imaging (MRI) proved to be the diagnostic test of choice. The addition of magnetic resonance angiogram (MRA) obviates the need for angiography. Our attempts at tumor embolization failed. CONCLUSION: An intersulcal splitting approach to the lateral ventricular trigone, combined with a perioperative external ventricular drainage, may be of value in the avoidance of symptomatic subdural effusions.

Cerebral Angiography↗

The role of endoscopic choroid plexus coagulation in the management of hydrocephalus.

Endoscopic choroid plexus coagulation has been used for the treatment of hydrocephalus at this unit for the past 20 years, and 156 operations have been performed on 116 patients. These patients were analyzed retrospectively to determine the rate of long-term clinical control of hydrocephalus, factors associated with successful control, change in ventricular size after surgery, and rate of surgical complications. Data were found for 104 patients with a median age at surgery of 5 months (range, 1 wk-30 yr) and a mean follow-up of 10.5 years. Control of hydrocephalus by choroid plexus coagulation was found to be best in children with communicating hydrocephalus and a slow to moderate rate of increase in head circumference (18 of 28, 64% long-term control), whereas those who presented with tense fontanels and rapidly progressive hydrocephalus had the lowest rate of success. Overall, 36 of 104 (35%) achieved long-term control without cerebrospinal fluid shunts. The ventricular size was not significantly reduced by choroid plexus coagulation (ventricular index before and after surgery, 0.64 and 0.58, respectively; P = 0.13), although sulcal markings became more prominent in all successfully treated patients, indicating reduced intracranial pressure. There were no deaths resulting from surgery, and serious morbidity was low. Eight patients developed infections (five meningitis and three implant infections). Other complications included postoperative fits (two patients), respiratory arrest in a premature infant (one patient), low-pressure state (one patient), ventricular drain displacement or blockage (eight patients), subdural effusion (one patient), and intraoperative minor ventricular bleeding, forcing abandonment of the procedure (two patients).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Retinal diseases with primary pulmonary hypertension].

BACKGROUND: Primary pulmonary hypertension (PPH) is a rare but fatal disease that results in right heart failure from idiopathic occlusion of the pulmonary arteries. Patients develop stagnation of venous pressure. PPH occurs most commonly in young and middle-aged women and the mean survival time from onset of symptoms is 2-3 years. The aetiology of PPH is unknown, although familial occurrence of the disease suggests a genetic predisposition. Pulmonary transplantation is reserved for patients who fail medical therapy. Pathophysiologically, PPH is a functional and structural alteration of the pulmonary blood vessels. METHODS: The authors retrospectively reviewed the clinical records of three patients (mean age 34 years) diagnosed with PPH and retinal diseases. RESULTS: All three patients developed central retinal vein occlusion (CRVO). One patient developed a neovascular glaucoma and on the other eye a serous retinal detachment with uveal effusion. A few months after the central vein obstruction was diagnosed two patients died as a result of PPH. CONCLUSIONS: The elevated venous pressure found in PPH is responsible for decreased choroidal perfusion and reduced venous blood outflow from the eye. This would explain the clinical findings of serous retinal detachment and central retinal vein occlusion in these patients.

Adult↗

Can chronic bulbar hypotony be responsible for uveal effusion? Report of two cases.

We describe two patients with a unilateral uveal effusion syndrome characterized by spontaneous ciliochoroidal and retinal detachment, shallow anterior chamber, poorly reactive pupil, and bulbar hypotony. A space-occupying substance of high molecular weight (sodium hyaluronate), injected into the anterior chamber, raised the intraocular pressure of these patients to 24 mm Hg. Within 24 to 72 hours the uvea flattened, and later the retina settled completely. Satisfactory clinical stability has continued in the follow-up period. Because a rheologic study of the aqueous humor revealed hyposecretion and hypotony in the apparently unaffected fellow eye, it is suggested that a primary bulbar hypotony can trigger a uveal effusion syndrome in an otherwise normal-sized eye with no structural abnormality.

Aged↗

Uveal effusion syndrome complicated by anterior ischemic optic neuropathy.

We report on a case of idiopathic uveal effusion syndrome complicated by AION. To our knowledge such an association hasn't been previously described. We suggest that scleral thickening caused obstruction of vortex veins followed by uveal effusion and compression of posterior ciliary arteries within their intrascleral tract, leading to AION. Nevertheless is can't be excluded that AION was the result of mechanical compression on ciliary vessels of optic disc by choroidal detachment.

Anti-Inflammatory Agents↗

Trisomy 21: 91% detection rate using second-trimester ultrasound markers.

OBJECTIVES: To examine cardiovascular and non-cardiovascular prenatal ultrasound markers and determine which markers physicians of varying skill levels could use to identify fetuses with trisomy 21. METHODS: Eighty second-trimester fetuses with trisomy 21 and 2000 controls underwent real-time plus color Doppler examination of cardiovascular and non-cardiovascular systems followed by amniocentesis. Non-cardiac markers were central nervous system malformations (CNS); choroid plexus cysts (CPC); abnormal nuchal skin fold (NSF); hyperechoic bowel (HB); and pyelectasis (PY). Cardiac markers consisted of ventricular septal defect, right-to-left chamber disproportion (RL); tricuspid regurgitation; mitral regurgitation (MR); pericardial effusion; and outflow tract abnormalities (OFT). Multinomial logistic regression was used to identify interactivity between the markers. Logistic regression was utilized to identify which combinations of markers significantly contributed to the identification of fetuses with trisomy 21 and to compute the likelihood ratio. RESULTS: All but three markers (CPC, MR, OFT) contributed significantly to the identification of 91% of fetuses with trisomy 21 with a false-positive rate of 14%. When only non-cardiovascular markers were examined, all but CPC contributed to the identification of 60% of fetuses with trisomy 21 with a false-positive rate of 5.9%. Combining right-to-left chamber disproportion with CNS, NSF, HB and PY identified 75% of fetuses with trisomy 21 with a false-positive rate of 6.4%. All markers were independent predictors of trisomy 21 except RL and NSF. CONCLUSION: Ultrasound can detect between 60 and 91% of fetuses with trisomy 21 depending upon which markers are selected for evaluation.

Adult↗

[Behavior of a Trypanosoma brucei strain (STIB 348C) in mice. 3. Histopathological findings in the terminal stage of infection].

Histopathological findings in the terminal stage of the infection of mice and rats with different variants of the Trypanosoma brucei brucei stock are described. Mice infected with mild variants showed after 12-149 days intense trypanosome colonization of the interstitial connective tissue, especially of the heart muscle, pancreas and choroid plexus, with severe tissue destructions, especially in the pancreas. With longer duration of the infection, round cell infiltrations of the leptomeninx and around intracerebral vessels developed. The alterations were the same in animals dying with high and low parasitemias. Some animals died with continuous bleeding from the tip of the tail, with serious effusions and massive edema or with generalized bacterial infection. The lymphatic organs showed intense reactive alterations, trypanosomes were only rarely found in lymph nodes. In the liver large fields of lymphatic and myeloid cells were seen, sometimes necroses developed. The kidneys showed marked deposition of eosinophilic material in the glomeruli and precipitation of proteinaceous material in dilated tubules. - Rats infected with mild trypanosomes exhibited intense colonization of the heart by trypanosomes, in some places many trypanosomes in lymph nodes, few trypanosomes in the pancreas. They died after a long phase of high parasitemia.--After infection with virulent parasites which killed the animals with high parasitemia in a few days, mice and rats had single small foci of trypanosome colonization of the interstitial connective tissue of heart, pancreas and choroid plexus as well as of the loose connective tissue in the hilum of the kidney and around lymph nodes.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Choroidal melanomas: correlation of NMR spectroscopy and MR imaging.

Six freshly enucleated, unfixed human eyes with choroidal melanomas were imaged on a 1.4-T superconducting magnetic resonance (MR) imaging system. Immediately thereafter the eyes were sectioned, and tumor samples were removed for study on a variable-field (0.19-1.4 T) nuclear MR spectroscopy unit. Shorter T1 and T2 relaxation times were observed in those tumors with the greater concentrations of melanin. This is believed to result from the paramagnetic effect of radicals known to exist in melanin. High magnetic field MR imaging can enable one to distinguish between pigmented melanomas; proteinaceous effusions; fresh and subacute hematomas; and nonmelanotic tumors, including amelanotic melanomas; but may not enable melanotic melanoma to be distinguished from fat or amelanotic melanoma from other nonpigmented tumors.

Choroid Neoplasms↗

Childhood choroid plexus papillomas: operative complications.

METHODS: Eight children (below 12 years of age) operated on for intraventricular choroid plexus papilloma (CPP) were retrospectively reviewed to identify the factors responsible for their high postoperative morbidity and mortality. Seven of these patients were aged between 2 months and 2 years and 1 was aged 12 years. Six CPP lesions were in the lateral ventricles and the remaining 2 were in the anterior third ventricle. All children presented with features of raised intracranial pressure. Due to gross hydrocephalus with severe manifestations at admission two patients required CSF diversion before definitive surgery. RESULTS: Microsurgical excision of CPPs was achieved in 7 and near total removal of the tumor in an 8th child. Brain shift was noted during operation and was attributed to acute CSF drainage and/or tumor excision in all cases. External ventricular drainage was postoperatively placed in 2 patients, who ultimately required shunt installation. One child died during definitive surgery. Due to neurological deterioration 6 of the remaining 7 patients had a postoperative CT scan within a week of surgery. One had an uneventful recovery, and pneumocephalus and subdural effusion were found in all 6 scanned children. Pneumocephalus was significant enough in 4 of them to warrant a surgical evacuation. CONCLUSION: Acute CSF drainage leading to significant intraoperative brain shift, postoperative external ventricular drainage, pneumocephalus, subdural effusion, and persistent postoperative hydrocephalus were identified as chief factors for higher morbidity in these children.

Child↗

Ultrasound biomicroscopic analysis of transient shallow anterior chamber in Vogt-Koyanagi-Harada syndrome.

PURPOSE: To evaluate the mechanism of formation of the transient shallow anterior chamber in Vogt-Koyanagi-Harada syndrome. METHODS: Two patients with Vogt-Koyanagi-Harada syndrome with shallow anterior chambers were examined with an ultrasound biomicroscope. RESULTS: A ciliochoroidal detachment, which was not obvious on ophthalmoscopic examination, was clearly demonstrated in both patients by ultra-sound biomicroscopy. The detachment disappeared after systemic corticosteroid therapy. CONCLUSION: The shallowing of the anterior chamber in two patients with Vogt-Koyanagi-Harada syndrome was caused by suprachoroidal effusion secondary to inflammation of the uvea.

Anterior Chamber↗

Uveal effusion syndrome: clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology.

PURPOSE: To clarify clinical features and pathophysiology and to evaluate surgical outcome of subscleral sclerectomy for primary uveal effusion syndrome. DESIGN: Prospective, consecutive noncomparative case series. PARTICIPANTS: Nineteen eyes of 16 patients diagnosed with uveal effusion syndrome treated in our clinic between 1989 and 1998. METHODS: Patients were examined by routine ophthalmologic examinations, fluorescein and indocyanine green angiography; measurement of the axial length of the eyeball; magnetic resonance imaging; and echography. Subscleral sclerectomy (sclerectomy under the scleral flap) was performed at the equator on all patients. Histologic examination of excised sclera was carried out on all samples. Patients were followed for outcome over time. MAIN OUTCOME MEASURES: Reattachment of the choroid and retina with resolution of the serous fluid. RESULTS: Three subgroups were identified: In type 1, nanophthalmic eye; the eyeball is small (average axial length 16 mm) and high hypermetropic (average +16 diopters); in type 2, the eyeball size is normal (average axial length 21 mm) with small refractive error; and in type 3, the eyeball size is normal. Histologically, types 1 and 2 demonstrated abnormal sclera with disorganization of collagen fiber bundles and deposits of proteoglycans in the matrix, whereas type 3 showed normal sclera. Subscleral sclerectomy was effective for types 1 and 2, inducing postoperative resolution of the subretinal fluid. However, type 3 eyes were not helped by this technique. CONCLUSIONS: Primary uveal effusion syndrome is caused by abnormalities of the sclera and increased resistance to transscleral fluid outflow as subscleral sclerectomy is an effective treatment in types 1 and 2 only, correct preoperative classification is essential for early surgical management.

Adult↗

[Perforating pars plana sclerotomy in idiopathic uveal effusion syndrome].

PURPOSE: To evaluate anatomical and functional results of perforating pars plana sclerotomy in 2 patients with the idiopathic uveal effusion syndrome (IUES). METHODS: In two patients with IUES the perforating pars plana sclerotomy was performed. During the surgery samples of the suprachoroidal fluid for biochemical analysis of protein fractions were taken. RESULTS: During the follow-up period (average 4 years) the retina and the choroid attached, and the central visual acuity (VA) improved as well in both patients. CONCLUSION: In IUES, the conservative treatment is not effective. Perforating pars plana sclerotomy is treatment of choice and it had positive anatomical and functional effect in both our patients with IUES.

Adult↗

The genetic sonogram: its use in the detection of chromosomal abnormalities in fetuses of women of advanced maternal age.

Real-time and color Doppler ultrasound were used to examine 103 second trimester fetuses with abnormal chromosomes (trisomies 13, 18, 21 and sex aneuploidy =86; other =17) and 2000 controls from women of advanced maternal age who electively underwent genetic amniocentesis. Ten ultrasound markers were analyzed and likelihood ratios were computed for each abnormal ultrasound finding and for a normal ultrasound study if none of the ten markers were present. Abnormal ultrasound markers were present in 81% of fetuses with abnormal karyotypes. The false-positive rate was 13%. The likelihood ratios and the 5% and 95% confidence limits for each of the ultrasound markers were as follows: choroid plexus cyst(s) 1.5 (0.7-3.6); central nervous system abnormalities 16.2 (4.4-60.3); abnormal nuchal skin fold 20.9 (8.4-52.1); ventricular septal defect 8.3 (4.7-14.9); outflow tract defects of the heart 3.6 (0.9-14.6); right-to-left chamber disproportion of the heart 36.9 (14.4-94.5); pericardial effusion 7.2 (3.2-16.1); tricupsid regurgitation 4.7 (2.1-10.7); hyperechoic bowel 3.7 (1.8-7.7); and pyelectasis 2.7 (1.0-7.7). All ultrasound markers were independent of each other. The likelihood ratio following a normal ultrasound study was 0.20. Isolated ultrasound markers were present in 20.4% (n=21) of fetuses. When all markers were compared to non-cardiovascular markers, the detection rate for fetuses with a chromosomal abnormality decreased from 81% to 52% (p<0.01). Given the above data, the posterior risk following an ultrasound examination using the ultrasound markers evaluated in this study can be used to compute the risk for an abnormal karyotypes in women of advanced maternal age.

Adult↗

Uveal effusion in primary angle-closure glaucoma.

PURPOSE: To determine the prevalence of uveal effusion in acute and chronic primary angle-closure glaucoma (PACG) or primary angle closure (PAC) and to compare it with the prevalence in eyes with open-angle glaucoma (OAG) or ocular hypertension. DESIGN: Prospective consecutive case series. PARTICIPANTS: Five hundred one eyes of 351 consecutive patients with PAC and 156 eyes of 116 randomly selected primary OAG or ocular hypertension patients. The PAC group included 40 eyes of 35 patients with acute PACG and 30 unaffected fellow eyes, 39 eyes and 35 fellow eyes with a history of acute PACG, and 357 eyes with chronic PAC. METHODS: Ultrasound biomicroscopic examination was performed to diagnose uveal effusion and to measure anterior chamber depth (ACD). MAIN OUTCOME MEASURES: Presence of uveal effusion and ACD. RESULTS: Uveal effusion was demonstrated in 23 eyes (58%) with acute PACG and 7 fellow eys (23%) (chi2 = 8.17, P = 0.0043). Among eyes with chronic PAC, uveal effusion was present in 69 [corrected](14%[corrected]), a higher prevalence than was found in open-angle patients (2 eyes [1.3%]) (chi2 = 19.3, P<0.001). In the chronic PAC group, the ACD of phakic eyes with uveal effusion (1.92+/-0.42 mm) was significantly shallower than that of phakic eyes without effusion (2.06+/-0.32 mm) (P = 0.019). CONCLUSIONS: Uveal effusion diagnosed by ultrasound biomicroscopy is a special feature in PAC, and is prevalent in acute PACG. Uveal effusion in phakic eyes with PAC is associated with shallowing of ACD.

Acute Disease↗