Multifactorial dysphagia complicated by esophago-bronchial fistula

Dysphagia in an elderly patient necessitates urgent clinical evaluation to exclude the possibility of an underlying esophageal malignancy. Atherosclerotic aortic aneurysms are common in old age, but dysphagia aortica resulting from compression of the esophagus by an aortic aneurysm is a rare cause for dysphagia. Development of a malignant esophago-airway fistula can occur from a variety of tumors, the most common of which is esophageal cancer. A case of longstanding dysphagia resulting from dysphagia aortica later developing an esophageal malignancy complicated by esophago-bronchial fistula is outlined in this unique case report.     

Successful resection of esophageal carcinoma associated with double aortic arch: a case report

Double aortic arch (DAA) is an extremely rare vascular malformation which causes tracheal and esophageal compression, resulting in respiratory symptoms such as stridor and wheezing, or feeding problems such as dysphagia, usually during the first few months of life. In contrast, this disorder is rarely diagnosed in adults. We herein present an elderly case with thoracic esophageal carcinoma with DAA. To the best of our knowledge, this is only the second report of a successfully resected case of esophageal cancer associated with DAA in the English literature. Moreover, this is the first reported case of symptomatic DAA with esophageal carcinoma. Spiral computed tomography and three-dimensional reconstruction was very useful for preoperative assessment of the abnormal vessels.     

Intramural aortic dissection

We present 10 cases of intramural aortic dissection. The cases are all characterized by the presence of intramural haematoma without the presence of a patent false lumen. The radiological features and possible aetiologies are discussed. The key radiological finding is the presence of a hyperdense rim in the aortic wall on a non-contrast-enhanced computed tomography (CT) scan. In one case, a delayed diagnosis was made using magnetic resonance imaging (MRI). In a further case, the delayed development of a large aortic ulcer was demonstrated. Intramural aortic dissection has only recently been described in the radiological literature. The aetiology of this condition remains controversial. The imaging findings may be subtle and the diagnosis is still frequently being overlooked. We believe CT to be the primary diagnostic test for this condition, and its advantages over MRI and transoesophageal echocardiography (TOE) are discussed.     

Chronic portomesenteic venous thrombosis complicated by a high flow arteriovenous malformation presenting with gastrointestinal bleeding

Portomesenteric venous thrombosis is a rare but potentially life‐threatening condition. The presenting symptoms of chronic portomesenteric venous thrombosis are often non‐specific but may present with variceal bleeding. We present the first reported case of chronic portomesenteric venous thrombosis causing a high flow arteriovenous malformation that resulted in extensive gastrointestinal bleeding.     

Coarctation of the Aorta Late in Life,with Special Reference to a Case Diagnosed by an Unusual Radiological Method

Before surgical repair became available, the average life expectancy of a patient with a coarctation in the classical position in the thoracic aorta was 35 years, because of the risk of various potentially fatal complications, which are enumerated. The cases of three patients, living respectively to 70, 61 and 53 years, two with autopsy proof, are reported to emphasize that considerably longer survival is nevertheless possible, with the condition unrecognized, and so unoperated. The need to check the chest film of even an elderly hypertensive patient for possible rib notching is stressed. One accepted complication of a coarctation is subarachnoid haemorrhage from a ruptured cerebral aneurysm, and the case of the third patient reported illustrates that the possibility of such an associated aortic anomaly should not be forgotten when a hypertensive patient suffers such an episode. The method of trans-femoral catheter angiography employed routinely at the Royal Melbourne Hospital for the investigation of subarachnoid haemorrhage led to the diagnosis, in this case, of an unsuspected coarctation.     

95例肾母细胞瘤临床病理特点及预后因素分析

  目的  分析肾母细胞瘤临床病理特点及预后相关因素。  方法  对首都医科大学附属北京儿童医院2001年1月至2007年12月期间收治的95例肾母细胞瘤资料进行收集, 分析其预后相关因素。  结果  本组研究中, 男性61例(60%), 平均发病年龄为3.16岁, 最常见的症状为腹部包块, 92例接受了手术治疗, 其中88例行完整肾肿瘤切除。Ⅰ期病例占41%、Ⅲ期为25%。随访患儿的总体生存率为78.9%, 随访的中位时间间隔为76.7个月, 分期是与预后相关的主要因素; 1例马蹄肾肾母细胞瘤; 7例患儿合并其他畸形。  结论  本组病例85%的肾母细胞瘤患儿于5岁前起病, 常见的症状为腹部包块, 右侧略多于左侧。以Ⅰ、Ⅲ期为主, 病理分型以FH为主, 转移部位为肺和肝脏。肾母细胞瘤总体生存率为78.9%, 分期是与预后相关的主要因素。特殊类型的肾母细胞瘤为马蹄肾肾母细胞瘤, 可并发其他器官先天畸形。      

Large focal nodular hyperplasia and extrahepatic portosystemic shunt in a male patient: multi-modality imaging features

Congenital extrahepatic portocaval shunt is a rare condition that is described mostly in female patients. We report an unusual case of a young adult male patient with type 1 congenital extrahepatic portocaval shunt with associated development of a focal nodular hyperplasia on a background of regenerative nodules. With multi-slice CT utilisation, there is increased detection of portocaval malformation in asymptomatic patients. This congenital variant is clinically significant with associated development of hepatocellular lesions, hepatic dysfunction and/or encephalopathy.     

Non-Immune Hydrops Fetalis: Rapidity of Onset and Usefulness of Prenatal Ultrasonography

Non-immune hydrops fetalis (NIHF) has become more common than immune hydrops fetalis as a cause of fetal hydrops and its contribution to the total perinatal mortality rate has increased from 0.1% to 3.0% for the 10 years to 1979. A case is reported where an antenatal ultrasonograph performed within 24 hours of delivery showed hydrops was not present, however, at birth the infant was grossly hydropic and died despite intensive management. This case shows the rapidity of onset of NIHF and the devastating effect of this disorder. A review of two large series of NIHF revealed that in only 11.4% and 16.3% respectively, a significant uncorrectible associated major malformation may have been missed by antenatal ultrasonography. Hence, if prenatal ultrasonography fails to reveal a major malformation a viable fetus with NIHF should be regarded as salvageable. Pulmonary hypoplasia occurred in over 90% and is probably due to compression from serous cavity effusions. Thus to improve survival the ultrasonographer needs to watch for the development of serous cavity effusions so that a pregnancy complicated by NIHF can be terminated before the fetus develops pulmonary hypoplasia.     

Posterior urethral valves

Posterior urethral valves represent the most common post-vesical obstructive malformation. They affect the male gender and appear as intraluminal folds located immediately proximal to the verumontanum. One of the most credited pathogenetic theories considers them an anomalous insertion of mesonephric duct into the cloaca or an incomplete involution of plicae colliculi. At present, the diagnosis of posterior urethral valves is prenatal and the pattern is characterized by detrusor hypertrophy and more or less marked hydroureteronephrosis. Urinary tract disorders that accompany posterior urethral valves include moderate hydroureteronephrosis to severe functional impairment of the entire urinary tract with consequent renal failure. Treatment of posterior urethral valves consists in their resection. At present, with miniaturized endoscopes, valve fulguration is feasible also in newborn infants. In low-weight patients or in case of poor general condition, temporary external urinary bypass (e.g. cystostomy) is feasible.     

Primary pulmonary rhabdomyosarcoma in a child, with a review of literature

A cyst in the left lower lobe of the lung was removed from a 15-month-old girl and showed evidence suggesting a preexistent congenital cystic adenomatoid malformation; in addition a rhabdomyosarcoma was identified. Primitive mesenchyme and mesenchymal elements also were seen. The origin of the rhabdomyosarcoma is discussed. The findings in the present case are compared with those of all childhood pulmonary rhabdomyosarcomas reported in the literature. This report of a child with a coexistent congenital adenomatoid malformation and a rhabdomyosarcoma is the third of its kind in the literature.     

Abdominal Aortic Aneurysm with Perianeurysmal Fibrosis: Demonstration of Ureteric Obstruction by Computerized Tomography

When perianeurysmal fibrosis occurs in association with an abdominal aortic aneurysm it may produce ureteric obstruction and renal function impairment. Such a case is described and the pre-operative radiological evaluation of this condition is discussed. The role of computerized tomography is emphasized in its ability to provide accurate anatomical detail regarding the aneurysm, the extent of surrounding fibrosis and ureteric involvement within it.     

Proboscis lateralis

Proboscis lateralis is a rare craniofacial malformation for which no embryological basis has been established. The condition presents with a tubular, nose like structure arising from the medial canthal area or orbit roof. We describe here such a case that was surgically treated.     

Mesenteric infarction secondary to tumor emboli from primary aortic sarcoma. Guidelines for diagnosis and management

Primary aortic tumors are rare, difficult to diagnose, and often fatal. This case and a review of the literature identified aortic tumors as a potential source of emboli leading to acute mesenteric insufficiency. The case showed the efficacy of magnetic resonance imaging in diagnosing and determining the extent and location of an aortic tumor.     

Arteriovenous malformation of the choroid plexus

A case of a 55-year-old female with arteriovenous malformation (AVM) of the choroid plexus within the right ventricle is reported. Arteriovenous malformation of the choroid plexus is a rare occurence. The MR findings of this malformation are discussed.     

Metastatic signet ring cell gastric carcinoma presenting as an infrarenal aortic aneurysm

Metastases to liver, lungs, bone, and adrenal glands are common events in advanced gastric carcinoma. Occasionally, metastases to other parts of the body, such as the prostate gland [1] the gluteal muscle [2], or the cervix [3] are described. However, these are rare events in the natural history of the disease. We report an unusual case of a signet ring cell gastric carcinoma, initially presenting as an infrarenal aortic aneurysm. Following resection of the aneurysm, the spread of lymphangiosis carcinomatosa into the aortic wall and infiltration of signet ring cells into an adjacent lymph node were noted. The primary tumor, a signet ring cell gastric carcinoma, was detected by a subsequent esophago-gastro-duodenoscopy     

Isolated duodenal metastasis from breast carcinoma

Small bowel neoplasms comprise 0.1% of all malignancies. Of these one third are duodenal, mainly primary. Secondary involvement of the duodenum by other malignancies or enlarged para aortic nodes is common but isolated duodenal secondaries arising on account of haematogenous spread is rare. Our case of duodenal obstruction due to an isolated duodenal metastasis as a result of haematogenous spread is unique in the literature.     

Isolated Lymphangiomatous Polyp Nasopharynx in an Adult First Case Report in English Literature

Lymphangiomas are rare benign, hamartomatous, congenital malformations of the lymphatic system involving the skin and subcutaneous tissues of head, neck and oral cavity. Occasional adult onset cases occur, this condition is thought to be a developmental malformation of lymph vessels which have poor communication with normal lymph system. Most of these malformations are present at birth or appear within two years of life. 75 % of cases occur in head and neck area, submandibular and parotid being the most affected parts. Lymphangioma arising in nasopharynx and in an adult has not been reported in english literature. This prompted us to report the very first case of Lymphangioma Nasopharynx.     

Pyogenic abscess complicating a resolving cerebral haematoma secondary to a cavernous haemangioma: Computed tomography and magnetic resonance imaging findings

A case is discussed of a brain abscess complicating an intracerebral haemorrhage occurring in a cavernous haemangioma. A young child presented with focal seizures as a result of a large intracerebral haemorrhage, occurring in a cavernous haemangioma. The only clue to the underlying vascular malformation was the presence of an associated developmental venous anomaly. The case was complicated by the development of a brain abscess at the site of the intracranial haematoma. The CT and MRI findings are discussed.     

Primary sarcoma of the aorta associated with a vascular prosthesis: a case report

A case of a primary malignant fibrous histiocytoma of the aorta, arising at the site of a woven Dacron thoracic aortic graft is described. Hypertension was a prominent clinical feature. Compression and distortion of the aortic graft by tumor complicated later surgical repair of an adjacent aortic aneurysm, resulting in death. At autopsy, the tumor was shown to have arisen in a portion of aortic wall surrounding the graft material. Tumor emboli were present in several distal arteries, with complete occlusion of the right renal artery by invasive tumor embolus. Primary tumors of the aorta are rare; 16 such cases have been reported in the literature. Only 2 previous cases of tumors associated with vascular prostheses have been reported. The occurrence of tumor in this case adjacent to an aortic graft raises the possibility that the graft material played a role in tumor induction.     

Neurofibromatosis 2: Report of an Affected Kindred,with a Discussion of Imaging Strategy

Three cases of neurofibromatosis 2 occurring in one family are presented. Features distinguishing this condition from the more common neurofibromatosis 1 are discussed and the important role of radiological screening in case detection and follow-up is emphasised.