Chronically ill children's participation and health outcomes in shared decision-making: a scoping review.

Based on the United Nations Conventions on the Rights of the Child (CRC), it is a child's right to participate in all matters concerning its wellbeing. Little is known about chronically and/or critically ill children's participation in pediatric shared decision-making (SDM). We explored medical literature to see if and how these children participate in pediatric SDM. We searched relevant medical databases published between January 2008 and January 2020 for studies targeting children aged 4-18 years old, suffering from a chronic and/or critical disease. We found 9 relevant studies. SDM interventions mostly used were decision aids (n=8), questionnaires for caretakers/parents and children (n=4), and a SDM toolkit (n=2). Perceived involvement in SDM and knowledge increased amongst children, adolescents, and caretakers following these interventions. Decisional conflict measured using the 0-100 point DCS scale (higher scores indicate more decisional conflict) was reduced by 15.9 points in one study (p<0.01) and 17.8 points in another (95%CI: 13.3-22.9). Lower scores were associated with higher satisfaction with the decision aid by children, caretakers, and clinicians.Conclusion: Stakeholders should advocate initiatives to facilitate a child's participation preferences regarding pediatric SDM since decision support tools help chronically ill children to be more involved in SDM as they increase the children's knowledge and satisfaction and reduce decisional conflicts. What is Known: • Decision aids can help improve participation, knowledge, satisfaction, and health outcomes. • Quality and consistency of the information exchange impact quality and outcome of SDM. What is New: • Depending on a child's age, evolving capacities, and communication and participation preferences, more evidence is needed on which tools are suitable for chronically ill children to ensure their preferred participation in pediatric SDM. • Pediatricians adopt healthcare SDM tools and techniques that do not always take into account that a child's right to participate in pediatric SDM including the tendency to use interventions that are not specifically designed for pediatrics.

Surgical repair of orbital fractures: how soon after trauma?

PURPOSE: To determine the results of surgical exploration soon after orbital trauma in comparison with surgery half a year or longer after trauma. METHODS: A retrospective study was done on 41 patients with motility disorders after trauma. Full ophthalmologic and orthoptic investigation was done in all patients. CT-scans and/or X-ray photographs were also made. The 41 patients were divided into two groups. One group of 13 patients did not need surgical repair. One group of 28 patients needed an operation. RESULTS: In 13 patients there was an orbital fracture but no surgical repair was needed. In 28 patients surgical repair was done. In 8 patients the operation was performed half a year or longer after trauma, 15 patients underwent surgery within two weeks after trauma, and 5 patients within two months. CONCLUSION: The best final results were obtained when the operation was done soon after trauma; however, the results in the patients who were operated late were fairly good too.

[111In-DTPA-D-Phe1] octreotide scintigraphy in thyroidal and orbital Graves' disease: a parameter for disease activity?

Visualization of malignant lymphomas and granulomatous disease is possible by [111In-DTPA-D-Phe1]octreotide scintigraphy through binding of the radioligand to somatostatin receptors on activated leukocytes. Because thyroidal and orbital tissues are infiltrated by activated leukocytes in Graves' disease, a cross-sectional study to visualize disease activity with [111In-DTPA-D-Phe1]octreotide scintigraphy was performed. A correlation between thyroidal [111In-DTPA-D-Phe1]octreotide accumulation and free T4 (disease expression) and thyroid binding-inhibiting immunoglobulins (disease activity) is present in untreated hyperthyroid Graves' disease. There is also a correlation between orbital [111In-DTPA-D-Phe1]octreotide uptake and the clinical activity score (disease activity) and total eye score (disease expression), respectively, in Graves' orbitopathy. Visualization of thyroidal and orbital Graves' disease is feasible, but further investigation is necessary to establish the role of [111In-DTPA-D-Phe1]octreotide scintigraphy in representing disease activity and expression and in predicting therapeutical outcome.

Transantral orbital decompression for Graves' disease.

Seventy-five patients with Graves' disease have been treated by transantral orbital decompression. In the first post-operative month the average reduction in proptosis was 3 mm. In the years following the operation this reduction increased to an average of 4.5 mm. In 32% of the patients without diplopia before surgery, the diplopia that developed afterwards did not disappear, 83% of them were successfully treated by extraocular muscle surgery. Seventy per cent of the patients experienced immediate post-operative improvement of visual acuity. Only three patients remained with anaesthesia of the infra-orbital nerve. A total of 65% of the patients found the operation procedure beneficial while 76% were satisfied with the ophthalmological result. We conclude, that transantral orbital decompression, though with moderate morbidity, gives good results in patients with the orbital complications of Graves' disease.

Intracavitary brachytherapy of cystic craniopharyngiomas.

Visual function, endocrinological status, and radiological outcome are reported in 31 patients harboring a cystic craniopharyngioma, who underwent 35 intracavitary brachytherapy procedures with yttrium-90. In 26 of these patients intracavitary brachytherapy was the primary treatment. The follow-up period ranged from 2 to 80 months (41 +/- 22 months, mean +/- standard deviation). Five patients died from tumor-related causes. Endocrine recovery was modest. Prior to brachytherapy, visual acuity was diminished in 38 eyes and field defects were present in 46. Funduscopy before treatment revealed optic atrophy in 47% of the eyes. Visual acuity improved in 29% of the eyes studied, remained stable in 13%, and deteriorated in 58%. Visual field defects improved in 28% of the eyes studied, remained stable in 20%, and deteriorated in 52%. The possible causes for deterioration in visual function are discussed. Complete resolution of 10 cysts was noted. In 12 patients the size of the cyst decreased; however, in three of these patients new cyst formation took place. The cyst size stabilized in six cases and increased in three. Although there is still a substantial degree of visual function deterioration following intracavitary brachytherapy, morbidity is otherwise low, making this treatment modality a reasonable alternative to craniotomy.

Mifepristone (RU 486) treatment of meningiomas.

Meningiomas are common brain tumours which are generally benign, well circumscribed and slow growing. In a minority of patients complete surgical removal is not possible and re-growth of tumour tissue is a major clinical problem. Most meningiomas contain progesterone receptors. The anti-progestational drug mifepristone (RU 486) binds to these receptors. Ten patients were treated with 12 recurrent or primary "inoperable" meningiomas, all of whom had shown recent neuroradiological and/or ophthalmological evidence of tumour growth. They received 200 mg mifepristone daily for 12 months. Most patients initially had complaints of nausea, vomiting and/or tiredness. In four patients prednisone (7.5 mg/day) was given after which these side-effects subsided. CT scan analysis of tumour size, showed progression of growth of five meningiomas in four patients, stable disease in three patients with three tumours and regression of four tumours in three patients. A decrease in the complaints of headache and an improved general well being was observed in five patients. Two patients died during the treatment period from unrelated causes. Mifepristone treatment resulted in control of tumour growth (= stable disease) in six of 10 patients who had shown recent evidence of tumour growth. In three of these six patients consistent tumour shrinkage was observed.

PIP: Meningiomas are common brain tumors which are generally benign, well circumscribed, and slow growing. In a minority of patients, complete surgical removal is not possible and regrowth of tumor tissue is a major clinical problem. Most meningiomas contain progesterone receptors and the antiprogestational drug mifepristone (RU 486) binds to these receptors. 10 patients were treated with 12 recurrent or primary inoperable meningiomas, all of whom had shown recent neuroradiological and/or ophthalmological evidence of tumor growth. They received 200 mg mifepristone daily for 12 months. Most patients initially had complaints of nausea, vomiting, and/or tiredness. In 4 patients, prednisone (7.5 mg/day) was given, after which these side effects subsided. CT scan analysis of tumor size showed a progression of growth of 5 meningiomas in 4 patients, stable disease in 3 with 3 tumors, and regression of 3 tumors in 3 patients. A decrease in the complaints of headache and an improved general well being was observed in 5 patients. 2 patients died during the treatment period from unrelated causes. Mifepristone treatment resulted in control of tumor growth (stable disease) in 6 of 10 patients who had shown recent evidence of tumor growth. In 3 of these 6 patients, consistent tumor shrinkage was seen.

Influence of age on the management of blow-out fractures of the orbital floor.

This study concerns 50 patients with blow-out fractures of the orbital floor, including 15 children, and was designed to evaluate the influence of age on clinical presentation and postoperative results. Fourteen of the 15 children were found to have a trap-door fracture. This type of fracture was not found in adults, who usually present with a large "open-door" fracture. In trap-door fractures, orbital tissues are liable to become trapped and even strangulated. It is therefore suggested that young patients with severely restricted eyeball motility, an unequivocal positive forced duction test, and findings indicating blow-out fracture of the orbital floor on CT, should undergo operative treatment as soon as possible after injury. A "wait and see" policy, keeping the patient under observation, seems to be appropriate for blow-out fractures in adults. Surgical treatment is recommended only in those adult patients who demonstrate impairment of vertical eyeball motility within the mainfield of view after the haemorrhage and oedema have resolved and in whom change in motility is no longer seen and Hertel measurements have stabilized.

Ten cases of optic vasculitis.

Optic disc vasculitis is an idiopathic, usually benign condition in young healthy adults. Hayreh suggested that the condition is to be divided into two types: type 1, with optic disc edema and a benign course as the dominant features and type 2, a condition resembling a central retinal vein (CRV) occlusion with a variable outcome depending on the extent and the site of the lesion. Differential diagnosis with other causes of one-sided papilledema (type 1) and other vascular conditions (type 2) is very difficult but important. We suggest performing extensive ophthalmological and neurological examinations to exclude major diseases. In this article we will give a review of the literature and a description of the ten patients with optic disc vasculitis who were seen in our clinic between January 1983 and August 1986.

Influence of transsphenoidal hypophysectomy on visual deficit due to a pituitary tumour.

The effect of transsphenoidal hypophysectomy is presented in 60 patients with visual impairment due to a pituitary adenoma, derived from a total series of 194 patients. The preoperative visual acuity and visual fields were compared with those one year after operation. The visual field loss was estimated using a semi-quantitative method. Seventy-two eyes had diminished visual acuity, and this improved in 45 eyes (63%). Visual field loss was present in 102 eyes and this improved in 71 eyes (70%). Improvement of either visual acuity and/or visual field was found in 84 eyes (78%). In 16 patients (27%) vision became normal after operation. Improvement of the visual acuity was usually accompanied by an improvement in the visual fields.

Radiotherapy of severe ophthalmic Graves' disease.

The effects of orbital irradiation on Ophthalmic Graves' Disease (OGD) were evaluated in 24 patients. All patients were irradiated after a median duration of eye symptoms of 12 months (range 3-36 months). Irradiation therapy was performed for loss of visual acuity, progressive diplopia and/or keratitis due to recent progression of eye symptoms. During a follow up period of 2-4.5 years, in 11 patients after irradiation, a mean decrease in proptosis oculi of 5.1 mm was found while 8 of these patients showed a mean increase in visual acuity of 0.26. Apparently, a decrease of activity of the inflammatory process in retrobulbar tissues can be achieved after irradiation, over a longer period of time in many patients. Furthermore concomitantly administered corticosteroids could be tapered off completely in all patients. Treatment of severe OGD of recent onset with irradiation, in combination with a short course of prednisone is very effective. This study also shows that prednisone treatment alone is not very successful because of the high recurrence rate of eye sings after decrease of the dose and the many side effects of the drug.

Electrodiagnostics of the tilted disc syndrome.

In five patients with a tilted disc syndrome the visually evoke potentials were examined by means of pattern stimulation. It was hoped that by such an examination this syndrome could be distinguished from a chiasma syndrome, since it is known that compression of the anterior visual pathways causes delayed responses, whereas defects or atrophies generally do not. In 4 patients, however, delayed responses were found and they were not found in only one patient.

Downward displacement of the eye resulting from orbital emphysema (case report).

A case is reported of unusual downward displacement of the eye, occurring in a patient with fractures of the orbital floor and rim. On the basis of the clinical, roentgenological and computerized tomographic scan findings this is interpreted as resulting from a subperiosteal pneumatocoele above the eye ball.

Pattern EPS in endocrine orbitopathy.

Since delayed pattern evoked potentials (EPs) are a sensitive symptom of demyelination or compression of the optic nerve, a group of 33 patients with endocrine orbitopathy were examined in order to see whether or not, in this disease too, damage to the optic nerve could be detected at an early stage. Below a visual acuity of 0.4, all patients had delayed responses. Most interesting was the group of patients with normal visual acuity and delayed responses. Before conclusions can be drawn this group will have to be enlarged and followed up.

Differential diagnostic aspects between optic neurititis and chiasm tumors.

Two patients with a pituitary tumor were first considered to have an optic neuritis from the clinical picture. The electrophysiological examination with VECPs gave results which did not offer a definite differentiation between both diseases, whereas perimetry and X-ray examination were decisive.

Visually evoked cortical potentials in the evaluation of homonymous and bitemporal visual field defects.

Visually evoked cortical potentials were studied in six patients with a homonymous and six with a bitemporal hemianopia by presenting a pattern-reversal stimulus separately to a temporal or nasal retinal area and by recording the responses from leads over the hemispheres. Homonymous visual field defects are characterized by a reduction of VECPs from the affected hemisphere. The disturbance of VECPs in bitemporal hemianopia is more serious, since the fibres from both retinal halves may be damaged by a chiasm tumour.