Idiopathic retroperitoneal fibrosis: the case for nonsurgical treatment

OBJECTIVE: To review a 10-year experience of medical treatment for idiopathic retroperitoneal fibrosis (RPF), a rare condition of unknown causes, which may obstruct the ureter and for which treatment includes ureterolysis and relief of obstruction, and medical treatment with steroids with or without immunosuppressive medication. PATIENTS AND METHODS: Of 31 patients with RPF treated between 1996 and 2004, 28 had idiopathic disease. Ureteric stents were used to relieve the obstruction in all patients, 12 had previous nephrostomies, 16 were given steroids and only one was treated with ureterolysis. RESULTS: In all, 28 patients were followed for a mean of 51.17 months; the symptoms were relieved in all cases. Renal function, where impaired, improved dramatically in all patients. Fifteen patients (54%) are free of stents and medication after a mean (range) follow-up of 60.9 (24-110) months. CONCLUSION: Idiopathic RPF responds well to conservative management consisting of relief of urinary tract obstruction and steroids.     

Giant mediastinal lymphangioma in a neonate: Report of a case

We report herein the case of a male neonate with a giant cystic lymphangioma confined to the bilateral anterior mediastinum. He developed very severe respiratory distress on the 8th day of life due to the mediastinal mass, and almost total excision of the bilateral mass was performed on the 12th day of life through a right axillar thoracotomy. The pathological diagnosis was cystic lymphangioma. Postoperatively, right phrenic nerve dysfunction was evident, for which diaphragmatic plication could only be performed 5 weeks after the initial surgery due to the prolonged respiratory support. In the 4 years since undergoing surgery the child has shown no sign of recurrence and has not experienced any further respiratory problems.     

Video-assisted thoracoscopic resection of a mediastinal cyst: Report of a case

Video-assisted endoscopic surgery has recently been expanding its potential, which is shown by our report herein describing the case of a 23-year-old woman for whom successful thoracoscopic resection of a mediastinal cystic lesion was carried out. The patient's postoperative course was uneventful with minimal pain and a prompt recovery. Histological examination confirmed that the lesion was a benign cystic teratoma. We believe that video-assisted thoracoscopic surgery will become the standard procedure for most mediastinal cystic lesions in the future.     

Postoperative chylothorax following partial resection of mediastinal lymphangioma: Report of a case

We report herein the rare case of a 20-year-old man in whom a mediastinal lymphangioma was incidentally detected by a chest roentgenogram taken during a routine health examination. Both computed tomography and magnetic resonance imaging confirmed a mass measuring 3×7 cm in diameter in the left anterior mediastinum. A thoracoscopic exploration was done, which confirmed a diagnosis of mediastinal lymphangioma, and 3 days later a sternotomy was performed. However, the tumor could not be completely extirpated due to partial invasion. Following the thoracoscopic procedure, a chylous discharge developed which was difficult to treat conservatively and he continued to drain 700–1,000 ml of chyle daily 2 weeks following the tumor extirpation. Therefore, a right thoracotomy with ligation of the thoracic duct was performed which resolved the chylothorax. The patient remains well without any regrowth of the regional tumor 9 months after his operation.     

Familial neurilemmomatosis: Report of a case

We report herein a case of familial neurilemmomatosis seen in a 16-year-old girl and her 38-year-old mother. The girl presented to us with an intrathoracic vagal neurilemmoma, as well as neurilemmomas on the right fifth intercostal nerve and in the serratus anterior muscle. Two years after these tumors were resected, bilateral acoustic neuromas and multiple brain stem tumors appeared. Her mother was being treated simultaneously at another hospital for multiple neurilemmomas, including bilateral acoustic neurilemmomas, suggesting the possibility that neurilemmomatosis is an autosomal dominantly inherited disorder.     

Thoracic epidural catheter in the management of a child with an anterior mediastinal mass: a case report and literature review

We describe a case of an anterior mediastinal mass compressing the right main bronchus that required a biopsy through a thoracotomy incision. The anesthetic management of these patients is associated with several risks and challenges, including potential airway compression and cardiovascular collapse. Inhalation induction and maintenance of spontaneous respiration is recommended to preserve normal transpulmonary pressure gradient and improve flow through conducting airways. We placed a thoracic epidural catheter under general anesthesia as the main analgesic technique in order to maintain spontaneous breathing. The use of regional anesthesia, especially continuous epidurals in pediatric cardiothoracic anesthesia have many theoretical advantages including attenuation of the neuroendocrine response, facilitation of rapid extubation and improved ventilatory mechanics secondary to decreased narcotic requirements. The absolute risk of nerve injury and epidural hematoma for this procedure is unknown and hard to define in this patient population. We reviewed multiple studies and case reports addressing its safety and reported side effects. Finally, we emphasize that a thoracic epidural anesthesia is a reasonable choice that can be applied carefully in special situations even for children under general anesthesia.     

Ectopic mediastinal thyroid removed by U-VATS approach. A case report

IntroductionEctopic thyroid tissue is a rare entity, and accounts for approximately 1% of all mediastinal tumours. It is a differential diagnosis of the mediastinum tumors or metastatic deposits from an orthotopic gland, as well as other benign or malignant masses. Although most cases are asymptomatic and discovered incidently by imaging, symptoms related to tumor size and its compression of adjacent structures may also appear which necessites explorations and lead to diagnosis.Case presentationThis is a 59-year-old women, followed for glaucoma and operated for bilateral congenital cataract reffered to our structure by the service of pnemology for a right laterotracheal mediastinal mass. The patient presented respiratory symptoms over four months, and the physical examination found patient in good condition with PS 0 and normal vital signs, a poor oral health was noticed. The CT scann showed a left basal opacity and a right laterotracheal mediastinal mass at the upper right mediastinum, pushing forward the superior vena cava and compressing the trachea on the contralateral side, with well-defined borders and without signs of infiltration of adjacent structure. The brochoscopy was perfomed which showed the yellowish granulous aspect and the pathophysiology revealed a pulmonary actinomycosis. The patient was treated with antibiotic based on parenteral infusion of penicillin G at 20 million / day for 6 weeks relayed by oral administration of 3 g / day for 3 months with a good response and the left basal opacity disappeared on the CT control but the mediastinal mass persisted. After multidisciplinary concertation, the mediastinoscopy was perfomed and has revealed an ectopic thyroid which was removed by Uniportal Videoassisted Thoracoscopic Surgery (U-VATS) approach.DiscussionThe first case of ectopic thyroid gland was described by Hickman in 1869, since a few cases have been reported by the literature. Its prevalence is about 1 per 100 000–300 000 people, rising to 1 per 4000–8000 patients with thyroid disease. The main techniques indicated in the management of undetermined lesions of the anterior mediastinum, are midline exploratory sternotomy, anterior lateral thoracotomy and VATS. U-VATS has demonstrated its feasibility and safety compared to conventional techniques by several advantages.ConclusionEctopic mediastinal thyroid is an unusual presentation of thyroid pathology. Complete surgical resection remains a therapeutic and a key diagnosis. The aim of this study is to prouve the feasibility, efficiency and efficacity of U-VATS approach as minimally invasive thoracic surgery for mediastinal mass resection.     

Idiopathic retroperitoneal fibrosis associated with IgG4-positive-plasmacyte infiltrations and idiopathic chronic pancreatitis

Idiopathic retroperitoneal fibrosis (IRPF) is an inflammatory fibrosclerosing condition, leading to renal failure by obstruction of the ureters. Idiopathic chronic pancreatitis associated with marked inflammatory infiltrates has recently been referred to as autoimmune pancreatitis (AIP), and infiltrating plasmacytes carrying immunoglobulin-gamma type 4 (IgG4) are relevant to its pathogenesis. The case is described herein of IRPF associated with subclinical pancreatitis that was most probably AIP in a 70-year-old man. Biopsy specimens of the retroperitoneal pseudotumor revealed a marked lymphoplasmacytic infiltration with dense fibrosis. Infiltrating plasma cells were immunoreactive for anti-IgG4 antibodies. Subsequent systemic examinations showed an extremely elevated serum IgG4 level and pancreatitis concordant with AIP. Following oral steroid administration, the serum IgG4 level normalized, although the appearance of the pseudotumor did not alter. Some AIP cases have been associated with idiopathic fibrosclerosing disorders including IRPF, but histological evidence of IgG4-related IRPF has rarely been provided.     

Idiopathic pulmonary fibrosis and anesthesia: The possible risk of oxygen administration

We studied the effects of a low oxygen concentration (less than 50%) during the induction of and the emergence from general anesthesia on the postoperative course of patients with idiopathic pulmonary fibrosis (IPF). Fifteen of 30 patients who had open lung biopsies between May 1990 and March 1994 were anesthetized with a low FIO₂ (<0.50) (Group A), while the other 15 patients received 100% oxygen (Group B). To evaluate the postoperative state, we used the ratio of the postoperative to the preoperative serum level of lactate dehydrogenase (post/pre LDH) and the number of days oxygen assistance was needed after operation. In addition, the following risk factors were also considered: age over 60 years, heavy smoking (a smoking index of more than 600), and a serum level of preoperative LDH higher than 400IU/I. The post/pre LDH was similar in the two groups (Group A 1.060±0.148; Group B 1.079±0.177, not significant). Group A patients needed fewer days of oxygen assistance after operation than did Group B patients (5.6±4.1vs 12.8±12.3;P=0.0426). The number of days using oxygen assistance after operation was: in the elderly, 5.0±1.7 in Group A, 11.8±2.0 in Group B (P=0.0236); in heavy smokers, 5.2±1.2 in Group A, 11.5±2.2 in Group B (P=0.0383); and in the high LDH groups, 4.7±1.1 in Group A, 19.0±7.5 in Group B (P=0.0483). We thus conclude that even such a short exposure to highly concentrated oxygen may affect the postoperative course. Therefore, anesthesia with low oxygen inhalation is recommended because it is simple and poses no risk to the patients.     

Tumor-Forming Idiopathic Retroperitoneal Fibrosis: Report of a Case

Idiopathic retroperitoneal fibrosis (IRF) is characterized by the progressive proliferation of connective tissue, but it rarely results in the formation of a mass. Herein, we report a rare case of tumor-forming IRF. A 76-year-old woman was referred to our hospital after a tumor in the right retroperitoneum was found by ultrasonography and computed tomography. Magnetic resonance imaging showed a 5 × 8 × 5-cm irregularly shaped tumor, lying adjacent to the right kidney, with a high-intensity T1-weighted image and a high-intensity T2-weighted image. Hormonal levels were within normal limits. Surgery was performed because of the possibility of an adrenal cancer. The tumor was firm, measured 7 × 8 × 4cm, and weighed 115g. The pathological diagnosis was retroperitoneal fibrosis. It is very difficult to distinguish tumor-forming IRF from malignancy. Several examinations, including needle aspiration cytology and biopsy, are necessary for the diagnosis and treatment of this disease.     

Mediastinal extraadrenal myelolipoma: Report of a case

We herein report a case of surgically resected mediastinal extraadrenal myelolipoma. Myelolipoma is an uncommon tumor composed of adipose tissue and normal hematopoietic elements, and is most often found in the adrenal glands. We could find only five such cases of mediastinal myelolipoma in the English literature.     

Anaesthetic management of an infant with anterior mediastinal mass

A substantial mediastinal mass in a small infant can create a dilemma regarding the safest mode of airway management. To ensure safety at all times, we adopted one lung ventilation for fear of compression of the carina and/or both main bronchi. Anaesthesia was maintained at a very light plane by the use of local nerve blocks to secure the airway and epidural analgesia for surgery until the tumour was mobilized.     

特发性腹膜后纤维化27例诊治分析

目的探讨特发性腹膜后纤维化(IRF)的临床特点及诊治原则。方法回顾性分析近8年收治的27例IRF患者临床资料。结果腹痛、腰痛、腹膜后包块及输尿管梗阻为主要临床表现。影像学检查为主要诊断手段。术前诊断正确率为85 2%。21例合并输尿管梗阻,行输尿管松解、大网膜包裹移位术; 2例合并肾动脉狭窄,行肾动脉松解术、IRF包块切除术; 1例合并肠系膜上动脉压迫,行松解术;另3例无梗阻,仅做活检。术后1例死亡, 26例临床治愈。结论IRF无特异性临床表现,术前诊断主要依据影像学检查。治疗主要解除压迫症状。     

同胞兄弟特发性腹膜后纤维化2例报告及文献复习

目的:提高对腹膜后纤维化疾病的认识。方法:回顾性分析2例同胞兄弟特发性腹膜后纤维化的临床资料,并结合文献复习对其病因、诊断和治疗等进行归纳。结果:弟弟首先发病,就诊时病理提示为晚期腹膜后纤维化伴有严重的肾功能损害,药物和手术治疗效果欠佳,通过肾造瘘维持肾功能,预后不良;哥哥随后发病,出现不典型的临床症状,由于有弟弟的相关病史而获得早期诊断和手术治疗,得到治愈。结论:腹膜后纤维化早期缺乏特异性临床表现,影像学的特征和病理表现有助于明确诊断和选择治疗方案。早期诊断疗效好,晚期患者应保护肾功能,提高生活质量,并做好长期随访。     

Aberrant retrotracheal goiter: Report of a case

A case of intrathoracic retrotracheal goiter in a 40-year-old woman is herein described. A computed tomographic scan, magnetic resonance imaging and endoscopical ultrasonography all confirmed the existence of a retrotracheal encapsulated lesion, which was suspected to be aberrant mediastinal goiter. Radioiodine scintigraphy, however, demonstrated little uptake in the mass. A large (7×6×4cm), encapsulated adenomatous goiter was then easily removed via a right thoracic approach.     

Spontaneous non-traumatic mediastinal hematoma associated with oral anticoagulant therapy: A case report and literature review

IntroductionMediastinal hematoma is usually caused by thoracic trauma or a ruptured aortic aneurysm. Spontaneous non-traumatic mediastinal hematomas are rare but potentially life-threatening conditions that can occur in patients taking anticoagulants.Presentation of caseWe report a case of 72-year-old man with a massive mediastinal hematoma associated with anticoagulant therapy. He had complained of acute chest discomfort and subsequent tarry diarrhea. Because he had been taking warfarin for paroxysmal atrial fibrillation, an upper gastrointestinal hemorrhage was initially suspected, but no bleeding was detected by upper endoscopy. A computed tomography scan revealed a massive posterior mediastinal hematoma and markedly compressed surrounding structures. The compression of the left atrium caused a congested lung and exacerbated respiratory and hemodynamic status despite conservative therapy. Therefore, we surgically removed the hematoma. Immediately after removal, the respiratory and hemodynamic conditions improved, and the postoperative course was uneventful.DiscussionSpontaneous mediastinal hematoma is rare but can occur in patients who are administered anticoagulants regardless of the therapeutic level of anticoagulation. Although conservative therapy is commonly effective, active surgical intervention should be considered for cases in which the hematoma is symptomatic or conservative therapy is ineffective.ConclusionTo facilitate prompt and proper management, clinicians should be aware of this condition as a potential complication of anticoagulant therapy.     

Anterior mediastinal mass in a pregnant patient: Anesthetic management and considerations

Patients with anterior mediastinal masses are recognized to be at risk for cardiorespiratory compromise when general anesthesia is induced.^1,2 Likewise, pregnancy has a widely known constellation of potential complications that confront the anesthesiologist. The combination of both problems in a single patient presents an unusual anesthetic challenge. The following is a case report of a pregnant patient with a large, symptomatic anterior mediastinal mass who required general anesthesia for a diagnostic procedure before definitive therapy could be initiated.     

前纵隔肿瘤的围术期麻醉管理

背景 前纵隔肿瘤对循环、呼吸影响重大,给手术麻醉带来极大挑战. 目的 为前纵隔肿瘤手术麻醉方案的制定和后续研究提供参考. 内容 对近年前纵隔肿瘤手术麻醉相关的病例进行回顾分析. 趋向 全身麻醉风险分级为“不安全”的前纵隔肿瘤患者应尽量避免全身麻醉,无法避免者,应在充分评估及准备下实施,术前在局部麻醉下行股动静脉穿刺,备体外循环是有必要的.全身麻醉诱导时,应在最适体位下缓慢进行,保持自主呼吸,只有当插管顺利且确保在气管内时,才可使用肌松剂.术中突发通气困难,在纤维支气管镜引导定位失败后,应紧急建立体外循环.术后拔除气管导管应在充分评估气道后实施.