医源性膈疝MSCT多平面重组表现

目的 分析食管癌术后继发医源性膈疝(IDH)的MSCT多平面重组(MPR)表现,探讨其诊断价值.方法 回顾性分析2010年1月至2014年3月间食管癌术后继发IDH 16例.将其CT号随机混编入其他50例食管癌术后无食管裂孔(EH)扩大患者队列,由2名不知研究目的从事腹部影像诊断的高年资医师分别就横断位和MPR图像进行判断.对不同医师、不同方法判断结果及影像表现差异采用x2检验.结果 16例IDH中3例局限性于EH,横断位2例呈假“肠套叠”征,1例呈肠扭转表现;MPR直观显示肠管挤入EH顶压胸腔胃呈“壁贴壁征”.13例超出EH范围,横断位表现为胸腔内肠管于各方向汇聚于EH处胸腔胃,其中,前外、后外及前后外侧分别为9例、5例和2例,前外侧明显多于前后外侧(x2 =6.79,P＜0.05),2例并肠系膜扭转;MPR直观显示胸腔内管肠及其系膜血管通过EH与腹腔相应结构连通.由2名医师横断位提示IGH诊断分别为3例和4例,MPR全部提示IDH诊断,均显著高于横断位(x2=21.89,19.20,P值均＜0.05).结论 食管癌术后继发IDH横断位表现隐匿,仔细观察并及时辅以MPR,对诊断具有重要意义.     

Multi detector CT Imaging of Abdominal and Diaphragmatic Hernias: Pictorial Essay

Diagnosis of abdominal wall hernia is often a clinical problem, especially in occult or in obese patients. Multidetector CT is an accurate method of detecting various types of abdominal and diaphragmatic hernias. It clearly demonstrates the anatomical sites of hernial sac, its contents and possible complications.     

Developmental outcomes of children with congenital diaphragmatic hernia: A multicenter prospective study

Purpose

To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age.

Methods

This is a multicenter prospective study of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age.

Results

BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors.

Conclusion

CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays.     

Symptomatic Right-Sided Diaphragmatic Hernia in the Third Trimester of Pregnancy

Background:

Laparoscopic repair of incarcerated diaphragmatic hernias is widely recognized as both safe and effective. However, symptomatic diaphragmatic hernias encountered in the setting of pregnancy, while rare, present a significant surgical challenge. Furthermore, right-sided diaphragmatic hernias account for only 13% of cases. Here, we present a case in which a symptomatic, posterior right-sided diaphragmatic hernia, presenting in the later stages of pregnancy, was successfully repaired using a laparoscopic approach.

Methods:

Our patient is a 42-y-old gravid woman who, at 27 wk gestation, was admitted to the gynecology service with a 2-d history of right upper quadrant abdominal pain, right shoulder pain, abdominal distension, and obstipation.

Results:

Computed tomography of the chest demonstrated an incarcerated right diaphragmatic hernia. Surgical consultation was obtained, and the patient was taken to the operating room urgently for repair. Intraoperatively, the cecum was reduced and the diaphragm repaired primarily using a laparoscopic approach. The patient recovered well and was discharged home on postoperative day 8 with no complications to the patient or the pregnancy.

Conclusion:

Laparoscopic reduction and repair of symptomatic incarcerated diaphragmatic hernia can be safely performed in the third trimester of pregnancy.     

An unusual presentation of perforated appendicitis in epigastric region

INTRODUCTION

Atypical presentations of appendix have been reported including backache, left lower quadrant pain and groin pain from a strangulated femoral hernia containing the appendix. We report a case presenting an epigastric pain that was diagnosed after computed tomography as a perforated appendicitis on intestinal malrotation.

PRESENTATION OF CASE

A 27-year-old man was admitted with a three-day history of epigastric pain. Physical examination revealed tenderness and defense on palpation of epigastric region. There was a left subcostal incision with the history of diaphragmatic hernia repair when the patient was 3 days old. He had an intestinal malrotation with the cecum fixed at the epigastric region and the inflamed appendix extending beside the left lobe of liver.

DISCUSSION

While appendicitis is the most common abdominal disease requiring surgical intervention seen in the emergency room setting, intestinal malrotation is relatively uncommon. When patients with asymptomatic undiagnosed gastrointestinal malrotation clinically present with abdominal pain, accurate diagnosis and definitive therapy may be delayed, possibly increasing the risk of morbidity and mortality.

CONCLUSION

Atypical presentations of acute appendicitis should be kept in mind in patients with abdominal pain in emergency room especially in patients with previous childhood operation for diaphragmatic hernia.     

Exome sequencing identifies a recessive PIGN splice site mutation as a cause of syndromic Congenital Diaphragmatic Hernia

Using exome sequencing we identify a homozygous splice site mutation in the PIGN gene in a foetus with multiple congenital anomalies including bilateral diaphragmatic hernia, cardiovascular anomalies, segmental renal dysplasia, facial dysmorphism, cleft palate, and oligodactyly. This finding expands the phenotypic spectrum associated with homozygous loss of function mutations in PIGN, and adds further support for defective GPI anchor biosynthesis as a cause of developmental abnormalities. We demonstrate that exome sequencing is a valuable approach for the identification of a genetic cause in sporadic cases of multiple congenital anomalies (MCA) due to inherited mutations.     

Diaphragmatic hernia a rare cause of acute pancreatitis: Case report

Many complications are known to occur in association with a diaphragmatic hernia. Acute pancreatitis occurring in this situation is very rare. In this paper, we report a case and describe the radiographic features of this complication. We report an unusual case of acute pancreatitis complicating a neglected post-traumatic diaphragmatic hernia in a 30-year-old male. This patient had a history of an abdominal trauma 5 years ago, and arrived at the emergency room with epigastria and left chest pain and vomiting. Serum lipase was elevated. Acute pancreatitis could be considered as an exceptional complication of diaphragmatic hernia. It is a serious diagnostic and therapeutic challenge. The fundamental roles of CT are to determine the diaphragmatic defect, the abdominal content involving, the Balthazar scoring of pancreatitis, and the presence of local complications. Even if a conservative approach is preferred when facing a diagnosis of pancreatitis, timing of surgery should be carefully considered.     

左旋精氨酸甲酯对大鼠膈疝模型肺血管发育的影响

目的 通过对大鼠孕期一氧化氮(NO)合成的抑制,探讨一氧化氮/环磷酸鸟苷(NO/cGMP)通路在大鼠膈疝肺动脉高压发病中的意义及部分膈疝患者NO吸入效果不佳的原因.方法 随机取孕9.5 d SD大鼠13只,3只作为正常对照组(C组),给予橄榄油2 ml灌入胃内,10只予异草醚(nitrofen)橄榄油溶液(200 mg/只)灌入胃内.左旋精氨酸甲酯干预分小剂量组、大剂量组(分别为L-NAME 1组、L-NAME 2组)进行,每组各4只,自孕14 d开始分别皮下注射左旋精氨酸甲酯(L-NAME)溶液250 mg/kg/d、500 mg/kg/d至孕20 d,对照组(CL组)3只于同样时间段给予生理盐水注射.分析各组血管计数、中膜厚度百分比(MT%),同时对内皮细胞源性一氧化氮合成酶(eNOS)表达进行免疫组化检测.结果 L-NAME 1组、L-NAME 2组与CL组胎鼠成模率、生存率比较,差异无统计学意义;L-NAME 1组、L-NAME2组与CL组血管计数、MT%均较C组明显增高,但L-NAME 1组、L-NAME 2组与CL组之间相比,无显著差异;膈疝发生后,eNOS阳性表达率降低;使用L-NAME后eNOS阳性表达率进一步减低.结论 阻断NO/cGMP通路,膈疝胎鼠肺小动脉的结构未出现相应变化.NO/cGMP通路的变化仅为肺动脉压力改变的功能性因素之一,调节NO/cGMP通路无法独立影响肺动脉的压力.