Anaesthesia for caesarean section in a patient with Ehlers-Danlos syndrome associated with postural orthostatic tachycardia syndrome

We report the use of regional anaesthesia for elective caesarean section in a primigravida with hypermobility type Ehlers-Danlos syndrome (type III) associated with postural orthostatic tachycardia syndrome. Single-shot spinal anaesthesia was performed after failed epidural anaesthesia. This genotype, although generally associated with fewer antenatal and peripartum complications, is more likely than other types to display dysautonomia and orthostatic intolerance. This requires careful management for delivery.     

Laparoscopic management of superior mesenteric artery syndrome.

OBJECTIVES: The differential diagnosis of intestinal obstruction includes mechanical obstruction, obstruction secondary to systemic disease, and idiopathic intestinal pseudo-obstruction. The causes of these are extensive; however, the majority of cases involve a mechanical cause. Superior mesenteric artery syndrome (SMAS) is a rare and controversial form of mechanical obstruction with just over 300 well-defined cases described in the literature. The diagnosis is often difficult to establish, even after surgery. In addition, this syndrome sometimes may be managed conservatively, leaving a definitive diagnosis unproven. We describe herein 2 patients with SMAS successfully treated with laparoscopic duodenojejunostomy. METHODS: Two cases of SMAS occurred in young men ages 23 and 34. The workup included a consultation with a gastroenterologist, an upper gastrointestinal (GI) endoscopy, upper GI series with small bowel follow-through, computed tomography scan, ultrasound of the abdomen, and abdominal aortogram. This diagnosis was established after consultation with the surgeon and the gastroenterologist in each case. RESULTS: Laparoscopic duodenojejunostomy was performed in each case, and both patients have had complete resolution of their preoperative symptoms. CONCLUSIONS: A laparoscopic approach to the management of superior mesenteric artery syndrome is a reasonable and successful way of treating these patients.     

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目的 对肠系膜上动脉压迫综合征的病因、诊断及治疗进行分析和探讨。方法 收集1970年1月至2000年8月住院治疗，经X线钡餐检验或手术确认为肠系膜上动脉压迫综合征77例。结果 保守治疗38例，好转31例（81．6％），未愈和未治6例，死亡1例。手术治疗39例，痊愈35例（89．7％），好转4例。结论 肠系膜上动脉压迫综合征的诊断除应具有上腹胀痛、呕吐等典型症状外，主要的确诊手段为X线钡餐造影；治疗上首先采用保守治疗，无效者再行手术治疗。术式选择以采用十二指肠空肠侧侧吻合术效果较为明显（P＜0．01）。     

Laparoscopic treatment of superior mesenteric artery syndrome.

BACKGROUND: Superior mesenteric artery syndrome is caused by compression of the third portion of the duodenum by the superior mesenteric artery. Many disease states predispose one to this condition. METHODS: We present a case report of a young female patient who presented with gastro-duodenal obstruction from superior mesenteric artery syndrome and subsequently underwent surgical treatment with minimally invasive techniques. Pathophysiology of SMA syndrome is reviewed. RESULTS: The cause of superior mesenteric artery syndrome is variable but always results in duodenal obstruction. Surgery is one treatment option that is effective and can be performed laparoscopically. CONCLUSION: Laparoscopic duodenojejunostomy is an acceptable method of treating superior mesenteric artery syndrome.     

Laparoscopic Ladd's procedure for superior mesenteric artery syndrome

Superior mesenteric artery (SMA) syndrome is an uncommon condition where the third portion of the duodenum is compressed and obstructed between the SMA and the aorta. An otherwise healthy 17-year-old female presented with a 2-month history of postprandial abdominal pain and weight loss. Upper gastrointestinal (UGI) series demonstrated SMA obstruction of the third portion of the duodenum. Despite nasojejunal tube feedings over 4 months with appropriate weight gain, symptoms continued and repeat UGI demonstrated persistent SMA syndrome. A laparoscopic Ladd's procedure served as definitive treatment. The steps of the procedure include mobilization of the Ligament of Treitz, mobilization of the right colon, complete derotation of the duodenum, delivery of the small bowel to the right upper quadrant, and appendectomy. Following the procedure, a postoperative UGI showed complete resolution of SMA compression of the duodenum. The patient had an uneventful postoperative course with immediate resolution of symptoms. She was discharged home tolerating a regular diet.     

肠系膜上动脉综合征的术式选择

目的　总结手术治疗肠系膜上动脉综合征的经验。方法　对近 10年手术治疗的 11例肠系膜上动脉综合征患者的病因、诊断、术前准备和手术方式进行回顾性分析。结果　 11例患者共施行手术 12次。行 Treitz韧带松解 1例 ,Billroth 式胃大部切除、十二指肠空肠侧侧吻合 3例 ,十二指肠空肠 Roux- Y吻合 4例 (其中 1例为胃空肠吻合术后再手术 ) ,十二指肠环形引流术 1例。全部病人随访均痊愈。结论　十二指肠空肠吻合术是治疗肠系膜上动脉综合征简单有效的手术方式     

Spontaneous rupture of the spleen in type IV Ehlers-Danlos syndrome: Report of a case

The vascular type of Ehlers-Danlos syndrome, type IV, is associated with severe complications, including arterial rupture and visceral perforation. However, to our knowledge, there has been only one previous report of splenic rupture caused by a spontaneous hemorrhage in type IV Ehlers-Danlos syndrome. We report another case of this uncommon complication, occurring in a 35-year-old woman who presented after the sudden onset of acute abdominal pain. Patients should be stabilized quickly in the intensive care unit and the most timesaving surgical techniques used. Moreover, tissues must be handled with great care intraoperatively in view of their extreme fragility. Despite prompt and appropriate treatment, the prognosis is often dismal.     

Laparoscopic duodenojejunostomy for superior mesenteric artery syndrome.

BACKGROUND: Superior mesenteric artery (Wilkie's) syndrome is a rare condition. Only 400 cases have been reported so far. The symptoms may be acute or chronic, the chronic form being more common. Vomiting is the most common symptom. About 15 causal factors have been found. Conservative management is the rule for acute cases. Surgery is indicated for chronic cases and failure of conservative management. Laparoscopy has been used in only 8 cases so far. CASE REPORT: We report the ninth case of superior mesenteric artery syndrome managed by laparoscopic duodenojejunostomy. The patient was a 14-year-old boy with chronic symptoms since childhood. The procedure was relatively straightforward. The case is being reported for its rarity and the possibility of laparoscopic management. DISCUSSION: Laparoscopic severing of Treitz's ligament is another surgical option, though gastrojejunostomy is of no use. Conservative management is useful only in acute cases. CONCLUSION: Duodenojejunostomy is the procedure of choice and is effective in 90% of patients. We conclude that it is very effective in this condition, especially laparoscopically.     

Superior mesenteric artery syndrome in an infant: case report and literature review

Superior mesenteric artery syndrome (SMAS) is an obstruction at the third portion of the duodenum by compression between the superior mesenteric artery and the aorta. In infancy, SMAS is extremely rare; and for its diagnosis, other duodenal obstructive diseases including congenital duodenal stenosis and intestinal malrotation must be ruled out. We present the case of a 7-month-old girl with frequent bilious vomiting after the resolution of acute gastroenteritis. Superior mesenteric artery syndrome was finally diagnosed at laparotomy, and duodenojejunostomy was performed. Vomiting disappeared postoperatively, and she gained weight. Although SMAS is an extremely rare syndrome in infants, it should be considered as a possible cause of incomplete duodenal obstruction.     

Superior mesenteric artery syndrome: Report of two patients and review of the literature

Superior mesenteric artery syndrome is a rare disease that should be considered in the differential diagnosis of upper abdominal pain, vomiting and weight loss. Compression of the third part of the duodenum between the superior mesenteric artery anteriorly and the aorta and the spinal cord posteriorly may result in high intestinal obstruction. Surgical treatment with duodenojejunostomy is indicated if proper conservative treatment has failed. Two patients are reported in the present case. One patient presented with acute surgical abdomen, which was an unusual presentation of superior mesenteric artery syndrome, while the second patient had classic features of the syndrome. Both patients were successfully treated with duodenojejunostomy. The pathophysiology, clinical presentation, investigation and management of this condition are discussed.      

Superior mesenteric artery syndrome following small bowel resection

Superior mesenteric artery syndrome is a rare cause of mechanical intestinal obstruction. It results from compression of the third part of the duodenum between the superior mesenteric artery and the aorta. It has been described in association with a variety of clinical conditions, including prolonged supine immobilization, application of a body cast, ileal pouch-anal anastomosis, abdominal aortic aneurysm, spinal surgery and severe weight loss. We present a case of superior mesenteric artery syndrome following resection of a primary adenocarcinoma of the small bowel.     

Superior mesenteric artery syndrome as a rare cause of duodenal obstruction

Superior mesenteric artery syndrome is a rare cause of duodenal obstruction. The syndrome can present as acute small bowel obstruction or intermittent compression symptoms such as postprandial epigastric pain, fullness or vomiting. The obstruction is caused by compression of the third part of the duodenum against the posterior structures by the narrow‐angled superior mesenteric artery. The diagnosis is easily confused with gastric outlet obstruction or proximal small bowel obstruction. A high index of clinical suspicion is crucial for diagnosis and computed tomography provides confirmatory evidence. We report two cases of superior mesenteric artery syndrome and a review of the literature for this condition.     

儿童肠系膜上动脉压迫综合征26例临床分析

目的 探讨儿童肠系膜上动脉压迫综合征(superior mesenteric artery syndrome,SMAS)的临床诊断和治疗方法.方法 结合文献复习对河南省人民医院小儿外科2013-03-2020-03间收治的26例SMAS患儿的临床资料进行回顾性分析.重点分析诊治方法和效果.结果 26例患儿中,男15例,...     

肠系膜上动脉压迫综合征的诊断和治疗

目的探讨肠系膜上动脉压迫综合征的病因、诊断和治疗方法。方法对1980年1月至2008年10月我院普外科收治的32例肠系膜上动脉压迫综合征的临床资料进行回顾性分析。结果本组均采用十二指肠空肠Roux-en-Y侧侧吻合加Treitz韧带松解术行手术治疗,术后病人症状均得到缓解,无死亡病例和并发症发生。结论肠系膜上动脉压迫综合征的主要确诊手段为X线钡餐造影;对保守治疗无效可采用手术治疗,十二指肠空肠侧侧Roux-en-Y吻合加Treitz韧带松解术为理想术式。     

顽固性便秘并发肠系膜上动脉综合征的危险因素和营养支持治疗效果分析

目的：探讨顽固性便秘患者并发肠系膜上动脉综合征（SMAS）的危险因素,并观察予以营养支持治疗的疗效。方法2008年6月至2012年12月间,南京军区南京总医院对连续收治的973例顽固性便秘患者作为研究队列,采用1∶4匹配的前瞻性巢式病例对照研究方法,确定顽固性便秘人群发生SMAS的危险因素;并评价营养支持治疗的疗效。结果973例顽固性便秘患者中确诊SMAS 26例,累计发病率为2．7％。多因素条件Logistic回归分析发现,体质量指数（BMI）低于18 kg/m2（OR＝2．89,95% CI：1.141~7.307）和结肠传输时间延长（OR＝3．57,95% CI：1.362~9.350）是发生SMAS的独立危险因素。经营养支持治疗,26例SMAS中有22例（84．6％）痊愈。与治疗前相比,SMAS患者的胃肠生活质量评分、Wexner便秘评分和营养指标（血清白蛋白、前清蛋白和纤维连接蛋白）均有明显改善（均P＜0.05）。结论 BMI低于18 kg/m2和结肠传输时间延长是顽固性便秘患者发生SMAS的危险因素。便秘并发SMAS应按病情和营养状况予以营养支持保守治疗,同时结合便秘对症治疗。     

肠系膜上动脉压迫综合征的诊疗分析

目的　总结肠系膜上动脉压迫综合征的诊断与治疗经验。方法　对我院 1 983年～2 0 0 2年诊治过的 1 6例病人的临床资料进行回顾性分析。结果　 1 6例病人均行钡餐造影检查 ,1 3例明确诊断 ;2例同时行腹部彩色多普勒超声确诊 ;另 2例同时行血管造影 ,明确诊断 ;1 6例病人均先行内科保守治疗 ,其中 8例因治疗无效而行手术治疗 ,包括十二指肠空肠Roux en Y吻合术 5例 ,十二指肠血管前移术 2例 ,胃大部切除、胃空肠吻合术 1例。术后病人全部随访 ,均痊愈。结论　钡餐造影、腹部彩色多普勒超声检查是肠系膜上动脉压迫综合征简单、可靠的诊断手段 ,十二指肠空肠吻合术是其有效、易行的手术方式     

肠系膜上动脉压迫综合征的外科治疗

目的 对肠系膜上动脉压迫综合征(superior mesenteric artery compressing syndrome,SMACS)的诊断及术式选择进行评价。方法 对河南省人民医院2000年1月至2010年12月期间收治的28例SMACS患者的临床资料进行回顾性分析。结果 本组病例均行X线钡餐检查,有23例呈典型的十二指肠水平段与升段交界处纵行受压迫征象（笔杆征）。手术治疗28例,其中包括Treitz韧带切断松解术4例,单纯胃空肠吻合术2例,Treitz韧带松解加十二指肠空肠Roux-en-Y吻合术11例,胃大部切除、胃空肠吻合术（BillrothⅡ式）7例及肠系膜上血管前十二指肠空肠吻合术4例。全部病例均治愈出院。结论 SMACS主要的确诊手段为X线钡餐造影。手术治疗效果以Treitz韧带松解加十二指肠空肠Roux-en-Y吻合术为最佳。     

Fournier gangrene associated with hyper IgE syndrome (Job syndrome)

Abstract:   We report a case of a 32-year-old man with hyper IgE syndrome (Job syndrome) who developed Fournier gangrene due to infectious multiple atheromas of the scrotal skin that progressed to the right groin and thigh. The patient required surgical debridement and subsequent skin grafting. This is a rare case of Fournier gangrene associated with hyper IgE syndrome (Job syndrome). When a patient without diabetes mellitus has repeated infections and atopic-like dermatitis, Job syndrome should be considered.     

Aneurysm of the inferior mesenteric artery associated with occlusion of the celiac axis and superior mesenteric artery

The authors report a case of aneurysm of the inferior mesenteric artery encountered in a 38-year-old man, associated with occlusion of the celiac axis, the superior mesenteric artery and the inferior mesenteric artery distal to the aneurysm. All three arteries were revascularized. In spite of failure in the bypass of the superior mesenteric artery, the patient remained symptom free until his demise four years later, from a probable myocardial infarction. Only 11 cases of aneurysms of the inferior mesenteric artery have been reported in the literature. The causes, diagnosis and treatment of these uncommon lesions are discussed. When occlusion of the celiac axis is associated with that of the superior mesenteric artery, a complete mesenteric revascularization should be attempted whenever possible.