老年痴呆患者遗嘱能力的研究进展

痴呆是一种发展病程不可逆的神经退行性病变,以损害患者的认知功能为核心。轻中度痴呆患者具有一定的认知功能及执行能力。遗嘱能力作为一项高级执行能力,因其跨越医学与法律的范畴引起了国外广大学者的关注。痴呆患者具有立遗嘱的权利,但其所立遗嘱是否有效值得探讨。目前国外对痴呆患者遗嘱能力评定标准、评估工具的研究逐渐增多,而我国对这方面的关注较少。本文通过回顾国内外老年痴呆患者遗嘱能力的相关文献并进行综述,以期为我国引进合理有效的遗嘱能力评定标准和评估工具提供依据,同时为保护我国痴呆患者合法权益、维护患者尊严、减少家庭和法律的纠纷提供借鉴。     

Vitrectomy in eyes with unsuspected retinoblastoma

OBJECTIVE: To analyze patient management and prognosis after vitrectomy in eyes with unsuspected retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Eleven consecutive patients who had undergone vitrectomy on an eye with unsuspected retinoblastoma. MAIN OUTCOME MEASURES: The two main outcome measures were ultimate patient management and the development of retinoblastoma metastasis. RESULTS: Of more than 900 consecutive patients with retinoblastoma managed on the Ocular Oncology Service at Wills Eye Hospital in Philadelphia, 11 (1%) had prior vitrectomy in an eye with viable tumor before referral to us for suspected retinoblastoma. The main preoperative diagnoses included vitreous hemorrhage in seven patients (64%), toxocariasis in two patients (18%), toxoplasmosis in one patient (9%), and endophthalmitis in one patient (9%). In no case was retinoblastoma suspected before vitrectomy. The mean patient age at vitrectomy was 6 years. Retinoblastoma was later suspected during vitrectomy in two patients (18%), on cytologic examination of the vitrectomy specimen in eight patients (73%), and after referral in one patient (9%). The mean interval between vitrectomy and referral to us was 23 days. On examination, the globe was classified as Reese-Ellsworth group Vb in all 11 patients (100%). Anterior chamber tumor cells were clinically visible in four eyes (36%), hyphema in two eyes (18%), and iris neovascularization in two eyes (18%). Retinoblastoma cells were visualized in the vitreous in seven eyes (64%) and not visualized in four eyes (36%) that had vitreous blood. Enucleation was necessary in all 11 patients (100%). Adjuvant treatment was delivered in 10 patients (91%), using orbital radiotherapy in nine patients (82%) and chemotherapy in nine patients (82%). Histopathologic evidence of retinoblastoma invasion was documented in the episclera (two eyes; 18%), anterior chamber (seven eyes; 64%), iris (five eyes; 45%), ciliary body (five eyes; 45%), choroid (three eyes; 27%), and optic nerve (four eyes; 36%; prelaminar, two eyes; postlaminar, two eyes). The vitrectomy ports, Tenon's fascia, cut end of the optic nerve, and orbit were free of tumor. Of the 10 patients who received prophylactic chemotherapy, radiotherapy, or both in addition to enucleation for prevention of retinoblastoma metastasis, none (0%) experienced metastasis or orbital recurrence during the mean follow-up of 7 years (range, 0.2-24 years) from the time of retinoblastoma diagnosis. However, one patient was referred to us after the development of metastatic retinoblastoma, and despite aggressive chemotherapy and radiotherapy after enucleation, died 24 months later. CONCLUSIONS: Retinoblastoma may present with atypical features such as vitreous hemorrhage or signs of vitreous inflammation, particularly in older children. Vitrectomy should be avoided in these cases until the possibility of underlying retinoblastoma is excluded. If vitrectomy is performed in an eye with unsuspected retinoblastoma, enucleation combined with adjuvant chemotherapy, radiotherapy, or both without delay is advised to prevent systemic tumor dissemination.     

An objective analysis of proliferative diabetic retinopathy before and after pars plana vitrectomy.

Nineteen eyes on which pars plana vitrectomy was performed were studied with fluorescein angiography. The degree of background diabetic retinopathy before and after surgical treatment appeared unchanged. The degree of proliferative retinopathy not only was less after surgical treatment, but continued to show regression with time.     

Clinical and follow-up studies of melanocytomas of the optic disc

Forty patients with melanocytoma of the optic disc were evaluated to determine their clinical variations and behavior. Follow-up examination of 1 to 19 years was obtained in 27 patients. Of the 27 lesions, four (15%) showed a slight increase in size, and one tumor (4%) decreased in size. The remaining 22 tumors (81%) have not yet demonstrated ophthalmoscopic changes. In spite of occasional growth, the melanocytoma is a benign tumor that requires no treatment.     

Circumscribed choroidal hemangioma : Clinical manifestations and factors predictive of visual outcome in 200 consecutive cases

PURPOSE: To review the clinical features and management of circumscribed choroidal hemangioma and determine factors predictive of poor visual outcome. DESIGN: Retrospective consecutive noncomparative interventional case series. PARTICIPANTS: Two hundred consecutive patients with circumscribed choroidal hemangioma. MAIN OUTCOME MEASURES: The main outcome measures were analyzed in 155 patients with follow-up of at least 3 months and included complete resolution of subretinal fluid, worsening of visual acuity (more than 2 Snellen lines), and poor final visual acuity (20/200 or worse). RESULTS: The patients were seen at a mean age of 45 years with symptoms of decreased visual acuity (81%), visual field defect (7%), metamorphopsia (3%), floaters (2%), progressive hypermetropia (1%), photopsia (1%), pain (1%), and no symptoms (6%). The referring diagnoses were choroidal hemangioma (29%), choroidal melanoma (29%), choroidal metastasis (9%), retinal detachment (6%), central serous chorioretinopathy (5%), and others. The tumor had a median base of 6.0 mm and median thickness of 3.0 mm. Secondary retinal detachment in the foveal region was present in 81% of the patients. Initial treatment included observation (51%), laser photocoagulation (44%), plaque radiotherapy (4%), external beam radiotherapy (1%), surgical repair of retinal detachment (1%), and enucleation for painful neovascular glaucoma (1%). Kaplan-Meier estimates revealed complete resolution of subretinal fluid in 60% patients at 5 years and 76% patients at 10 years follow-up. By multivariable analysis, clinical factors predictive of complete resolution of subretinal fluid included shorter duration of symptoms (P = 0.03) and inferior quadrant location of tumor (P = 0.001). At initial presentation, 82 of 155 (53%) patients had poor visual acuity (20/200 or worse), and 73 of 155 (47%) patients had good to moderate visual acuity (20/100 or better). Of those 82 patients with poor initial vision, poor final vision was found in 54% at 5 years and 80% at 10 years. Of the 73 patients with good to moderate initial vision, poor final vision was found in 12% at 5 years and 43% at 10 years. By multivariable analysis, clinical factors predictive of poor final visual acuity included poor initial visual acuity (P < 0.001), failure of previous laser photocoagulation before referral (P = 0.01), and tumor management with observation after referral (P = 0.02). Worsening of visual acuity (by more than 2 Snellen lines) was observed in 8% at 5 years and 28% at 10 years of those 82 patients who were initially seen with poor vision. Worsening of visual acuity was found in 10% at 5 years and 30% at 10 years of those 73 patients who initially were seen with good to moderate vision. CONCLUSIONS: Circumscribed choroidal hemangioma is a rare intraocular tumor. In 38% of cases, this tumor is initially misinterpreted before referral as choroidal melanoma or metastasis. Visual acuity is poor in more than 60% of patients at 10 years, despite successful control of associated subretinal fluid in 76% patients.     

Tubular apocrine adenoma of the eyelid – A case report and literature review

Tubular apocrine adenoma is a rare benign adnexal neoplasm most commonly identified in the scalp, composed of a dermal proliferation of apocrine tubules in a background of hyalinized stroma. Tubular apocrine adenoma can be a component of various sweat gland tumors and can also morphologically overlap with other sweat gland neoplasms. Isolated tubular apocrine adenoma arising in the glands of Moll is exceedingly rare, with only 4 previously reported cases. We present a 63-year-old male with tubular apocrine adenoma of the left upper eyelid, which recurred following initial incomplete excision. Although the lesion showed focal morphologic similarity to the apocrine variant of pleomorphic adenoma (chondroid syringoma), the diagnosis of tubular apocrine adenoma was supported by fluorescence in situ hybridization studies, which demonstrated absence of PLAG1 and HMGA2 gene rearrangements seen in pleomorphic adenoma. This case illustrates the clinical, microscopic and immunohistochemical features of tubular apocrine adenoma. The recent advances in our understanding of the molecular genetics of tubular apocrine adenoma and related tumors, and how these advances shape the evolving classification of sweat gland tumors are reviewed.     

Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors.

OBJECTIVE: To evaluate the clinical factors predictive for tumor recurrence and treatment complications in a large series of children who underwent plaque radiotherapy for retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: The participants included 141 children with retinoblastoma who were managed on the Oncology Service at Wills Eye Hospital with plaque radiotherapy between July 1976 and June 1999. MAIN OUTCOME MEASURES: Tumor recurrence and treatment complications. RESULTS: There were 208 tumors managed with plaque radiotherapy. The mean patient age at plaque treatment was 19 months. Prior treatment to the retinoblastoma of concern was delivered to 148 tumors (71%) and included various combinations of treatments such as intravenous chemoreduction, external beam radiotherapy, laser photocoagulation, thermotherapy, and cryotherapy. For 72 retinoblastomas (35%), more than one therapeutic method had failed to achieve tumor control before the use of plaque radiotherapy. Of the 208 retinoblastomas managed with plaque radiotherapy, Kaplan-Meier estimates of tumor control were 83% at 1 year and 79% at 5 years. Of the 60 tumors treated only with plaque radiotherapy (primary treatment), recurrence at 1 year was 12%. Of the 148 tumors treated after failure of other methods (secondary treatment), specific Kaplan-Meier estimates of tumor recurrence at 1 year was detected in 8% of tumors previously treated with chemoreduction, 25% of tumors previously treated with external beam radiotherapy, 34% tumors previously treated with both chemoreduction and external beam radiotherapy, and 8% of tumors previously treated with laser photocoagulation, thermotherapy, or cryotherapy (methods other than chemoreduction and external beam radiotherapy). Using multivariable analysis, the risks for tumor recurrence included the presence of tumor seeds in the vitreous, presence of subretinal tumor seeds, and increasing patient age. Using Kaplan-Meier estimates, radiation complications at 5 years of follow-up included nonproliferative retinopathy in 27%, proliferative retinopathy in 15%, maculopathy in 25%, papillopathy in 26%, cataract in 31%, glaucoma in 11%, and scleral necrosis in 0%. CONCLUSIONS: Plaque radiotherapy for retinoblastoma provides tumor control in 79% of cases at 5 years of follow-up. It is particularly useful for those tumors that fail treatment with chemoreduction, laser photocoagulation, thermotherapy, and cryotherapy. Tumors in young patients without vitreous or subretinal seeding show the best long-term control.