共同管综合征———胆、胰管异常汇合

对我院14年来2322例直接胆管造影所发现的27例AJPBD进行研究，27例中胆总管囊肿8例，胆石症9例，慢性胰腺炎4例，胆管炎3例，胆囊癌2例，胰体癌1例。临床表现为上腹痛（92．6％），黄疸（70．4％），发热（48．1％）或Charcot三联症（44.4％）。发病年龄3～60岁（36．9士17．8）。共同管长15～44mm（20.2士5．9）。据胆、胰管汇合形式作者将AJPBD分为三型。值得注意的是本组3例复杂型患病年龄均＜15岁。本组以胆、肠内引流手术效果满意。AJPBD与胆、胰疾病发生、发展密切相关，作者建议用“共同管综合征”来表述这一复杂病理现象。     

Invited. Fukuyama congenital muscular dystrophy: A neuroradiologic review

We reviewed neuroradiologic findings of Fukuyama congenital muscular dystrophy (FCMD) and correlated them with the known neuropathology. All patients showed thick and bumpy cortices with shallow sulci corresponding to polymicrogyria, and approximately half of the patients showed pachygyric cortex with smooth surface corresponding to type II lissencephaly. The two types of cortical dysplasias presented characteristic distributions: the former demonstrated frontal lobe involvement in all and parietotemporal lobe involvement in some, whereas the latter involved the temporo-occipital lobes. Most patients showed prolonged T1 and T2 signal in the white matter, which was indistinct in neonates and infrequently seen in adolescents. Cerebellar polymicrogyria depicted as disorganized cerebellar foliation accompanying cysts were found more than 90% of the patients. In conclusion, brain MRI demonstrates findings consistent with the known neuropathology of FCMD. The detection of the two types of cerebral cortical dysplasia with characteristic distribution and cerebellar abnormalities is helpful in the differential and early diagnosis.     

489例不良孕产史患者细胞遗传学分析

目的探讨染色体异常对生育的影响.方法对489例有自然流产、胎停育或生育畸形儿史的患者进行外周血淋巴细胞培养,G显带染色体核型分析.结果 489例患者中染色体核型异常24例, 异常率达4.9%, 其中有自然流产、胎停育史的患者核型异常占20例,有生育畸形儿史的患者核型异常占4例.染色体变异共21例,占4.3%.结论染色体异常是导致不良生育的重要影响因素, 应重视遗传学检查,进行优生优育指导.     

Amyotrophic cerebellar hypoplasia

Summary Lower motor neuron degeneration, cerebellar hypoplasia, atrophy of pons, olives, and cerebellum, sclerosis of thalamus and pallidum, and deficient myelination were found in a 2-months-old baby with laryngeal paralysis, mental retardation, progressive amyotrophy, and slow nerve conduction velocity. Such changes seem characteristic of an unusual syndrome previously referred to as cerebellar hypoplasia in Werdnig-Hoffmann disease, or anterior horn cell disease with pontocerebellar hypoplasia. Although the pathologic changes in lower motor neurons are indistinguishable from those in other cases of infantile spinal muscular atrophy, the consistent reproducibility of a complex pathologic pattern suggests that this is probably a manifestation of a separate disease process. The term amyotrophic cerebellar hypoplasia (ACH) is a convenient designation for the syndrome.Supported in part by National Institutes of Health grant no. RR75     

机器人手术系统在女性梗阻性生殖道畸形中的应用

目的:分析达芬奇机器人手术系统在女性梗阻性生殖道畸形中的临床应用效果.方法:回顾性分析空军军医大学第一附属医院2015年9月～2019年10月使用达芬奇机器人手术系统完成的梗阻性生殖道畸形手术患者7例的临床资料,统计分析不同生殖道畸形的分类、临床表现、手术方法、术后并发症、月经恢复情况和性生活满意度等.结果:7例患者中,宫颈发育异常者6例,阴道完全闭锁者4例,阴道下段闭锁者1例,合并子宫内膜异位症者6例,合并泌尿系统畸形者1例.所有患者均保留了有功能子宫,6例行宫颈成形术,5例行阴道成形术;所有患者均恢复规律月经,2例有性生活者性生活均满意.结论:对于梗阻性生殖道畸形的患者,达芬奇机器人手术系统能更好地重建生殖道解剖结构,保留有功能子宫.     

WARFARIN EMBRYOPATHY

A 34 year-old gravida 2 para 1 (1001) mother was on prophylactic anticoagulation therapy because of pulmonary emboli postabdominal surgery. The pregnancy was terminated at 25 weeks' gestation after ultrasound examination showed a mild hydrocephalus and a cardiac structural defect. Congenital abnormalities of the delivered fetus included nasal hypoplasia, earfold atresia, bilobled lungs, coarctation of the aorta, ventriculuar septal defect, gastroschisis, and radiographic skeletal stippling. The radiological and physical findings were consistent with warfarin embryopathy.     

经口咽入路显微手术治疗颅颈区畸形

目的 总结经口咽入路颅颈区畸形的显微手术治疗体会。方法 回顾30例颅颈区畸形病例的临床表现、术前准备、手术方法、术后效果及并发症，并结合文献进行分析。结果 本组30例颅颈区畸形，经行口咽入路显微手术后，随访3个月～4年，痊愈21例(70％)，好转6例(20％)。结论 采用经口咽入路显微外科手术是治疗颅颈区畸形的有效手段，术中良好的显露及镜下细心操作是降低并发症的关键。     

内耳畸形与耳蜗骨化患者的耳蜗植入

目的探讨先天性内耳畸形与耳蜗骨化患者耳蜗植入术的有关问题.方法对我院2002年10月～2004年2月间行耳蜗植入术的8例内耳畸形和2例耳蜗骨化的患者进行回顾性分析.结果4例大前庭水管综合征患者术中有外淋巴液搏动;3例Mondini畸形和1例共同腔畸形患者术中出现井喷;1例耳蜗部分骨化患者术中误将电极插入内听道后纠正;另一例耳蜗部分骨化患者植人短电极.1例Mondini畸形患者术后发生少量脑脊液耳鼻漏,保守治疗3月后痊愈,其余患者无并发症.所有患者均成功开机.结论对于内耳畸形或耳蜗骨化的患者,耳蜗植入术前详细的影像学评估,对术中困难的充分估计和正确、规范处理是手术成功的基本条件.     

Grief after termination of pregnancy due to fetal malformation

Termination of pregnancy for fetal malformation is a traumatic event which any woman finds hard to withstand and which entails the risk of severe and complicated grieving. This paper presents three cases illustrating the trauma and coping mechanisms. Grieving continued for over 6 months in all cases and included pathological anxiety and depression. We offer advice and counselling to such women.