Iniencephaly and long-term survival: a rare case report

Introduction Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine, and retroflexion position of the head is a very rare congenital abnormality of the fetus–newborn with a 0.1–10 of 10,000 prevalence. This abnormality’s prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders, diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal atresia. Discussion Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think that this patient presents a mild form of iniencephaly.     

Comparative early and midterm results of open juxtarenal and infrarenal aneurysm repair

Background and aims Since the introduction of endovascular aortic aneurysm repair (EVAR) for aortic aneurysms, the number of juxtarenal aortic aneurysms (JRA) has been growing steadily due to selection bias (neck morphology for EVAR). This case-match study compares the perioperative outcome and midterm results of suprarenally clamped JRA with infrarenal aortic aneurysms (AAA). Methods From 1997 to 2004, patients who received open surgery with suprarenal clamping for JRA were included in the study and compared to matched patients with infrarenal clamping (AAA). Measurements analyzed were the in-hospital mortality and morbidity. Midterm results were obtained through clinical investigation and magnetic resonance angiography imaging. Results Thirty-five patients (mean age, 68.4 years; 30 male and 5 female) received suprarenal cross-clamping for JRA. The overall in-hospital mortality for JRA and for the controls (AAA) with elective aortic repair was 4.5% (6.1% JRA; 3% AAA, p = 0.058). The morbidity of JRA was elevated according to the rate of pulmonary complications (p = 0.021) and the need for re-operation (p = 0.019). The mean follow-up time was 2.3 years (range, 8–96 months). At follow-up, 28 patients (80%) from the JRA group and 29 patients from the AAA group (82.9%) were alive. Conclusion Open aortic surgery for JRA with the need for suprarenal cross-clamping shows a slightly elevated in-hospital mortality rate without statistical significance and equal midterm mortality results in comparison with infrarenally clamped aortic aneurysms.     

Thyroid Artery Erosion by Esophageal Cancer: Management with Interventional Radiology

Management of upper gastrointestinal bleeding because of erosion of vessels by esophageal cancer may be challenging. We present herein the angiographic images of a 49-year-old patient who was admitted with massive bleeding from a tumor-eroded inferior thyroid artery. Attempts to control the bleeding by means of flexible endoscopy and insertion of a Sengstaken–Blakemore tube had failed. The diagnosis was impressively demonstrated by multislice computed tomography with intravenous contrast in the arterial phase and multiplanar reconstructions (computed tomography angiography) and by digital subtraction angiography. The bleeding was successfully treated with superselective catheterization and coiling of the eroded vessel.     

鼻胃管胃肠减压在择期腹部手术中的应用价值

鼻胃管胃肠减压曾被常规应用于择期和急诊的腹部手术后，目的是预防急性胃扩张的发生、治疗梗阻、降低吻合口压力等。但是，鼻胃管置入也会带来一些副作用，如呼吸道并发症、胃-食道反流、体液和电解质的丢失、声带的损伤，以及越来越被重视的患者的不适感。近年来的研究结果也对择期的腹部手术后常规应用鼻胃管胃肠减压提出不同的看法。现重新评价应用鼻胃管胃肠减压的理论基础及相关临床研究的结果，并对鼻胃管胃肠减压在择期腹部手术中的价值作一综述。     

Are common mutations of cystathionine β-synthase involved in the aetiology of neural tube defects?

Mildly elevated maternal plasma homocysteine (Hcy) levels (hyperhomocysteinemia) have recently been observed in some neural tube defect (NTD) pregnancies. Plasma levels of Hcy are governed by both genetic and nutritional factors and the aetiology of NTDs is also known to have both genetic and nutritional components. We therefore examined the frequency of relatively common mutations in the enzyme cystathionine β-synthase (CBS), which is one of the main enzymes that controls Hcy levels, in the NTD population. Neither the severely dysfunctional G307S CBS allele nor the recently reported 68 bp insertion/I278T CBS allele was observed at increased frequency in the cases relative to controls. We therefore conclude that loss of function CBS alleles do not account for a significant proportion of NTDs in Ireland.     

Fallopian tube carcinoma presenting as tubo-ovarian abscess: a report of two cases with literature review

Preoperative diagnosis of fallopian tube carcinoma is difficult, with fewer than 5% being diagnosed preoperatively. We describe tubal carcinoma, presenting as a tubo-ovarian abscess in two 47-year-old women. Both patients presented with abdominal pain, pelvic mass, and fever. Both patients were treated as having a tubo-ovarian abscess but failed to respond to therapy. During surgery a metastatic right tubal carcinoma was found. A definite operation was performed in both patients. Three additional cases of fallopian tube carcinoma, presenting as acute pelvic inflammatory disease, were found while reviewing the English literature. Actually all these three cases presented as tubo-ovarian abscess because of the existence of tender pelvic mass. Carcinoma of the fallopian tube should be considered in the differential diagnosis of tubo-ovarian abscess in those who failed to respond to a previously unreported clinical presentation.     

经皮扩张性气管切开术的临床应用

目的：探讨经皮扩张性气管切开术行气管切开及气道套管插入的方法与效果。方法：在内科ICU病房内，采用一步性扩张气管切开术，对35例患者进行气管切开，同时采用纤维支气管镜对其中l0例患者进行气道内检查。结果：按照操作规程，均完成了气管切开及气管套管的插入，平均操作时间为5．4min．所有患者在带管过程中及拔管后，未发现有气道狭窄的发生，无瘘管或瘘道形成，且创口愈合良好。结论：经皮扩张性气管切开术操作简便易行，值得临床推广使用。     

左双腔支气管导管管端位置与吸气峰压变化的关系

目的 观测无隆突钩双腔支气管导管(DLT)管端位置与吸气峰压(Ppeak)以及肺顺应性环形状改变的关系，探讨用Ppeak和顺应性环的变化评估DLT管端位置的可行性。方法 拟行右侧剖胸手术的成年患者32例，静脉诱导后插入左Mallinckrodt DLT，吸入氧化亚氮和地氟醚维持麻醉。按纤维支气管镜(FOB)确认DLT管端位置和通气方式将观测过程分为四个阶段：第一阶段(S1)，管端位置正确的双肺通气；第二阶段(S2)，管端位置正确的左侧单肺通气；第三阶段(S3)，管端插入左下支气管(置管过深)的左侧单肺通气；第四阶段(S。)，管端处在左支气管开口(置管偏浅)的左侧单肺通气。每阶段均机械通气15min。结果 回归方程预计插管深度与FOB检查符合者占71．9％。S2时Ppeak值比S1时增加50．8％，肺顺应性(Cdyn)值减少36．2％；S3时Ppeak值比S1时增加87．4％，Cdyn值减少56．8％。PV环曲线斜率向右明显移位，环体显著延长。结论 用无隆突钩DLT行肺隔离，在无FOB定位的条件时，联合应用听诊法、回归方程预计插管深度、动态监测气道峰压和P-V环的变化综合评估，可提高DLT管端的正确到位率。单肺通气的气道峰压超过双肺通气时的1．65倍，且气道峰压超过25cm H2O．应高度怀疑DLT管端发生过深移位。     

PRENATAL DIAGNOSIS Ⅰ. EARLY DIAGNOSIS OF OPEN NEURAL TUBE DEFECTS BY QUALITATIVE ACETYLCHOLINESTERASE ISOENZYME IN AMNIOTIC FLUID

The measurement of amniotic fluid (AF) acetylcholinesterase isoenzyme (AChEI) is a relatively new method for early diagnosis of open neural tube defects (NTDs). As quantitative methods are of unproven reliability at present, the authors used a high resolving power qualitative method-vertical slab polyaerylamide gel electrophoresis. The benefits of this technique are: simplicity of operation, accuracy, unsophisticated equipment, and easily available reagents. Combined results of 9 NTDs studies revealed that samples from early pregnancy gave more accurate results than those from late pregnancy.