目的探讨原发性骶前肿瘤的诊断与治疗方法。方法回顾性分析1979~2001年间收治的12例原发性骶前肿瘤患者的临床资料。结果直肠指诊、B超、CT检查阳性者分别为10/12、 12/12及6/6； 肿瘤完全切除者9例，部分切除者2例，肿瘤未能切除仅行隐性乙状结肠造口术1例。经病理组织学检查，12例中畸胎瘤4例，上皮样囊肿5例，恶性淋巴瘤1例，神经纤维瘤1例，神经纤维肉瘤1例； 8例术后平均随访14个月，治愈6例，好转2例,无手术死亡。结论原发性骶前肿瘤临床表现不典型，直肠指诊、B超及CT检查有助于诊断； 完全切除是治疗原发性骶前肿瘤的主要措施。
ObjectiveTo observe the clinical characteristics of primary retinitis pigmentosa (RP) complicated with glaucoma.MethodsA retrospective clinical study. From June 2008 to March 2020, the diagnosis of primary RP were included in the diagnosis confirmed by the eye examination of West China Hospital of Sichuan University included 4794 eyes of 2432 patients. Among them, 4679 eyes (97.2%, 2364/2432) were in 2364 cases with RP alone, and 115 eyes were in 68 cases with RP combined with glaucoma (2.80%, 68/2432). All affected eyes underwent best corrected visual acuity (BCVA) and intraocular pressure examination. The BCVA examination was carried out using the international standard visual acuity chart, which was converted into the logarithmic minimum angle of resolution (logMAR) visual acuity during statistics. The 67 eyes of 40 patients with RP and glaucoma with complete follow-up data were analyzed to observe the proportion of different glaucoma types, logMAR BCVA, intraocular pressure and other clinical characteristics, as well as treatment methods and post-treatment intraocular pressure control. After treatment, the intraocular pressure ≤21 mm Hg (1 mm Hg=0.133 kPa) was regarded as intraocular pressure (IOP) control; ＞21 mm Hg was regarded as uncontrolled IOP.ResultsAmong the 67 eyes of 40 cases with complete follow-up data, 5 cases (7 eyes) with primary open-angle glaucoma (10.45%, 7/67), 56 cases (58 eyes) with angle-closure glaucoma (ACG) (86.57%, 58/67), 4 cases (4 eyes) with neovascular glaucoma (5.97%, 4/67), 2 of them had both ACG and neovascular glaucoma. Among 58 ACG eyes, 17 eyes were acute ACG (25.37%, 17/67), 21 eyes were chronic ACG (31.34%, 21/67), and 2 eyes were suspicious angle closure (2.99%, 2/67), lens dislocation secondary to angle-closure glaucoma in 8 eyes (11.94%, 8/67), chronic angle-closure glaucoma after anti-glaucoma surgery, intraocular lens shift in 5 eyes (7.46%, 5/67), 5 eyes (7.46%, 5/67) secondary to glaucoma with true small eyeballs. The logMAR BCVA 3.50 of the affected eye,＜3.50-＞2.00, ≤2.00-≥1.30,＜1.30-＞1.00, ≤1.00-0.52,＜0.52 were 9 (13.43%, 9/67), 30 (44.78%, 30/ 67), 7 (10.45%, 7/67), 4 (5.97%, 4/67), 11 (16.42%, 11/67), 6 (8.96%, 6/67) eyes, which correspond to mean intraocular pressure were 32.31±11.67, 30.15±14.85, 28.17±13.19, 31.50±17.25, 18.71±8.85, 14.12±4.25 mm Hg. Among 67 eyes, 37eyes (55.22%, 37/67), 18eyes (26.86%, 18/67), and 6 (8.96%, 6/67) eyes underwent surgery, medication alone, and peripheral iris laser perforation treatment, respectively. The treatment of 6 eyes was abandoned (8.96%, 6/67). Malignant glaucoma occurred in 3 eyes (8.11%, 3/37) after the operation, all of which were after trabeculectomy of the ACG eye. After treatment, intraocular pressure was controlled in 37 eyes (55.22%, 37/67), 19 eyes were not controlled (28.36%, 19/67), and 11 eyes were lost to follow-up (16.42%, 11/67).ConclusionsThe incidence of glaucoma in patients with primary RP is 2.80%. ACG is more common, and the combined lens dislocation or intraocular lens shift is more common.
Objective To introduce a method of preoperative three-dimensional measurement by echocardiography to guide the surgical resection of hypertrophic obstructive cardiomyopathy (HOCM) and its long-term follow-up effect. MethodsBefore operation, each patient underwent transthoracic echocardiography to measure the length, width and thickness of diastolic ventricular septum hypertrophy on the long axis, short axis and four chamber sections, in order to establish three-dimensional measurement data of myocardial hypertrophy, and quantitatively estimate the location, depth and range of myocardium to be removed between 2014 and 2022 in our hospital. According to the quantitative data during operation, the hypertrophic myocardium of ventricular septum was resected to dredge the left ventricular outflow tract. ResultsForty-three patients were recruited, including 22 males and 21 females, aged 18-78 (49.2±5.1) years. Eighteen patietns underwent mitral valve surgery at the same time. All patients were satisfied with the relief of left ventricular outflow tract obstruction. Postoperative transesophageal echocardiography showed that the left ventricular outflow tract pressure gradient decreased significantly (94.2±28.1 mm Hg vs. 6.7±4.7 mm Hg, P<0.05). There was no ventricular septal perforation or complete atrioventricular block during the operation, and no one needed a secondary aorta-clamp for re-operation to remove hypertrophic myocardium again. Postoperative echocardiography showed that the mitral valve closed well or only had mild regurgitation, and the mitral systolic anterior motion sign basically disappeared. After 1.0-8.5 years of follow-up, the average pressure difference of left ventricular outflow tract remained below 10 mm Hg, and the clinical symptoms disappeared or improved significantly. Conclusion The quantitative prediction of the resection range of hypertrophic myocardium by three-dimensional measurement of preoperative echocardiography can accurately guide the surgical range of HOCM, avoid multiple blocking of aorta during operation, relieve left ventricular outflow tract obstruction to the greatest extent, and obtain better long-term results.