Barium studies in esophageal cicatricial pemphigoid

Background: Cicatricial pemphigoid (CP) (benign mucous membrane pemphigoid) is a rare, blistering disease of skin and mucous membrane. The disease rarely extends to involve the esophagus, and there are only a few cases reported in the radiological literature. The aims of this study were to document the frequency of esophageal involvement and to describe the findings on upper GI barium studies. Methods: A total of 197 patients with CP were seen at our institution from 1981 to 1991. The clinical and radiological findings of these patients were reviewed and compared with findings reported in the literature. Results: Esophageal involvement was documented in seven patients. Cervical esophageal webs were found in five of the seven patients. Two patients had single esophageal webs while three had multiple webs. Frank strictures of the esophagus were also seen in five patients. These were most common in the cervical esophagus, but strictures were also found in the mid and lower esophagus. Two of the strictures resulted in significant dysphagia and required multiple endoscopic dilatations. One of the dilatations was complicated by mucosal injury, and follow-up barium examination showed dissection of the esophageal mucosa from the cervical esophagus to the esophagogastric junction. One patient demonstrated intramural pseudodiverticulosis in the cervical esophagus. Functional disturbances demonstrated on barium studies included tracheal aspiration in two patients and nasopharyngeal reflux in three. Conclusions: CP involves the esophagus in approximately 5% of cases. The hypopharynx and cervical esophagus are most commonly involved, but any portion of the esophagus may be involved, and multiple levels of involvement may be seen. Cervical esophageal webs, often multiple or complex, are the most common appearance on barium studies, but frank strictures are also found. Secondary manifestations of esophageal involvement include nasopharyngeal reflux, tracheal aspiration, and intramural pseudodiverticulosis.     

Sonography of colonic diverticulitis

Sonographic findings in 16 patients with diverticulitis of the colon are described. The wall of the inflamed segment of the colon appeared hypoechoic and thickened. Maximum thickness of the wall ranged from 5 to 17 mm. Length of the most severely inflamed segment of the colon ranged from 6 to 9 cm. In addition, adjacent contiguous segments of the colon, less involved with inflammation, ranging in length from 7 to 15 cm, were demonstrated on sonographic examination of five patients. Long segments of the inflamed colon could be demonstrated by oblique scanning. The appearance of the mucosal reflections, intramural abscesses, and inflamed diverticula is described. Out of 16 patients with diverticulitis, seven patients had abscesses and extravasation of barium was seen in only two of these seven patients. Ultrasonography therefore is recommended prior to barium or water-soluble contrast enema examination in patients with suspected diverticulitis. In patients with appropriate clinical findings, sonographic diagnosis of diverticulitis can be made by demonstrating hypoechoic thickening of the wall of the colon, even in the absence of intramural or intraperitoneal abscess.     

Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) was once uniformly fatal. Therapeutic plasma exchange in combination with immunosuppressive and anti-platelet agents, however, have resulted in improved survival rates of greater than 80% for patients with TTP. In spite of aggressive plasma exchange and adjuvant therapy, a number of TTP patients are refractory to treatment. In addition, up to 40% of TTP patients who initially respond to therapy eventually relapse. Alternative therapies such as splenectomy have been used with varying degrees of success in refractory and relapsing TTP patients. The usefulness of splenectomy in preventing relapse of TTP or treating those patients who are refractory to plasma exchange remains controversial. We present a single institution's experience with 14 patients who underwent splenectomy for refractory (six patients) or relapsed (eight patients) TTP since 1984. In both patient groups, splenectomy induced stable long-term remissions. Six of six (100%) patients who were refractory to plasma exchange, survived to be discharged from the hospital, apparently free of disease. Four of eight patients (50%) who had a splenectomy for relapsing TTP went into a complete remission and had no further relapses of their disease. Moreover, in relapsing patients who failed to experience long-term remission, the relapse rate after splenectomy was 0.3 events per patient year compared to 1.0 events per patient year prior to splenectomy. We conclude that splenectomy is a reasonable treatment option for TTP patients refractory to standard plasma exchange therapy or who have experienced multiple and/or complicated relapses. We believe this is the first series that demonstrates efficacy of splenectomy in plasma exchange-refractory TTP.     

Intramural barium in ischemic colitis: a new radiographic finding.

Two cases of ischemic colitis are presented, demonstrating a new radiographic finding of intramural dissection of barium. Colonoscopic findings were compatible with that diagnosis. Deep, discrete ulcerations were observed during the healing process. Both patients recovered and prolonged retention of the intramural barium was seen.     

One-year follow-up of plasma exchange therapy in 14 patients with idiopathic thrombocytopenic purpura

Fourteen patients with idiopathic thrombocytopenic purpura (ITP) have been followed for one year after plasma exchange therapy. Exchange was performed prior to splenectomy in eight of nine patients with acute ITP and following splenectomy in five patients with chronic ITP. None with chronic ITP showed a response in platelet count as a result of exchange therapy. Four of the nine with acute ITP had poor responses and required splenectomy because of persistent severe thrombocytopenia. Three of these have responded completely and are in remission. One patient with acute ITP had an equivocal response, with a most recent platelet count of 93,000/microliter. The remaining four patients with acute ITP had prompt and complete responses and now have platelet counts above 100,000/microliter without steroid treatment. Although the good responses were temporally associated with the use of plasma but not albumin, the data are not sufficient to conclude that a plasma factor must be infused to obtain a satisfactory result. The overall response after one year was about that expected for acute ITP patients treated with prednisone and early splenectomy. Exchange plasmapheresis may be of value in decreasing the number of patients who require splenectomy, but a randomized, prospective study is needed to adequately assess this possibility.     

Computed tomographic angiography identification of intramural segments in anomalous coronary arteries with interarterial course

Certain coronary anomalies are associated with high risk features. We sought to determine the diagnostic accuracy of coronary computed tomographic angiography (CTA) in determining high-risk features, particularly intramural segments. Anomalous coronary arteries can be associated with adverse clinical events. Anomalous coronaries that course between the great vessels (interarterial) have been associated with sudden death. High-risk features of interarterial vessels described in the literature include; a slit-like orifice, acute angle of origin, and intramural segments (within the wall of the aorta). Although computed tomography (CT) findings of acute angle and slit like orifice have been described previously no prior evaluations regarding CT identification of an intramural segment have been reported. An intramural segment has distinct surgical management implications. All interarterial anomalous coronary arteries do not have an intramural segment. Since October 2004, 15 patients were diagnosed by CTA as having an anomalous coronary artery with an interarterial course, which were then confirmed by intraoperative examination of their coronary origins and course during aortic root/coronary artery surgery. The CTA images were retrospectively analyzed for the presence of high-risk features by a radiologist blinded to the surgical findings. Comparison of these findings was made to the findings at surgery. The anomalous coronary was the right coronary artery in 10 patients and the left coronary artery in 5. Eleven patients had an intramural segment identified at surgery. Pre-operative coronary CTA showed that all patients with an intramural course of the anomalous artery, had slit-like orifice, an acute angle of origin (mean 18.4 ± 3.4°), and an elliptical shaped cross-section throughout the intramural segment of the anomalous vessel. The average vessel height/width ratio for anomalous coronary vessels without an intramural segment was 1.03; compared to a ratio of 2.19 for anomalous vessels with an intramural segment (P = 0.003). Coronary CTA can identify an intramural segment of an anomalous interarterial coronary artery by its elliptical shape. Identifying an intramural segment has important clinical and surgical implications.     

Adult ileocolic intussusception secondary to a submucosal cecal lipoma

Intussusception is a relatively common cause of intestinal obstruction in children but a rare clinical entity in adults, representing fewer than 1% of intestinal obstructions in this patient population. We present a rare case of a 44-year-old female patient with intestinal obstruction due to ileocolic and colocolonic intussusception secondary to an intramural cecal lipoma. Diagnosis was made by barium enema and abdominal computed tomography and was confirmed by colonoscopy. After failure of conservative treatment, the patient underwent surgery.     

外伤性延迟性脾破裂的诊治探讨(附17例报道)

目的：探讨外伤性延迟性脾破裂的发病规律、临床特点、诊断和治疗方法。方法：结合国内外资料及本组病例进行回顾分析。结果：明确诊断13例，误诊为肝破裂1例，腹膜后血肿1例，肾挫裂伤1例，宫外孕破裂1例。16例手术治疗，1例非手术治疗。行脾切除术10例，其中保留副脾1例，脾切除加自体脾组织网膜内移植术1例，脾缝合修补术3例；脾部分切除术3例。治愈16例，死亡1例。结论：本病由于腹腔内出血与受伤时间间隔长，容易误诊。诊断除依靠病史、临床表现外，应及时进行腹腔穿刺、B超及CT检查。治疗以脾切除为主，可根据病情、脾破裂的程度以及是否有合并伤等情况采取保脾手术或非手术治疗。     

Radiographic evaluation of achalasia immediately after pneumatic dilatation with the Rigiflex dilator

Radiographic evaluation of the lower esophagus was done immediately after pneumatic dilatation using the Rigiflex dilator in 34 patients (24 men, 10 women; mean age, 55 years) with achalasia. The dilator was positioned across the esophagogastic junction using fluoroscopy and the balloon was inflated for 1 min. The esophagus was intubated and injected sequentially with water-soluble and barium contrast materials. Radiographic analysis included changes in the appearance of the caliber and contour of the esophagogastric junction, rate of esophageal emptying, and presence of complications. In 23 patients with predilatation esophagrams, the mean esophagogastric junction caliber increased from 4.7–7.6 mm following dilatation. The postdilatation esophagrams in 33 patients showed a smooth contour in 22 (67%) and immediate esophageal emptying in 26 (79%). Esophageal perforation occurred in one (3%) patient and intramural hematoma in one (3%). Clinical follow-up (mean, 7 months) was available in 29 patients and 23 (79%) had symptomatic improvement. Five of the six patients who did not improve clinically all had previous Heller myotomy, pneumatic dilatation, or both.     

Treatment of hairy cell leukemia with cladribine (2-chlorodeoxyadenosine)

Hairy cell leukemia is a rare lymphoproliferative disorder resistant to conventional chemotherapeutic agents. Recently, the purine analogue cladribine (2-chlorodeoxyadenosine, 2-CdA) was introduced for the treatment of this disease. We report on 14 patients with hairy cell leukemia who were treated with 2-CdA at our department between 1993 and 1997. The patients received a single cycle of 2-CdA at a dose of 0.07 or 0.09 mg/kg/day by continuous infusion, over a seven-day period. Five patients were previously untreated, while the others had received prior treatment with interferon-alpha (seven patients), interferon-alpha and splenectomy (one patient) or interferon-alpha, splenectomy and pentostatin (one patient). Six patients achieved complete remission, three a good partial response and three partial remission. Two patients did not respond to treatment and one of them died from septicemia in aplasia. Relapse of the disease occurred in two patients. Side effects such as fever (WHO grade 2) and/or neutropenia (WHO grade 4) were noted in eight patients. Thus, 2-CdA is an effective treatment of hairy cell leukemia that can induce long lasting remissions in both, previously treated and untreated patients.     

Invasive group B streptococcal infection in a patient with post splenectomy for hypersplenism secondary to liver cirrhosis and portal hypertension

BACKGROUND: Splenectomy in patients with liver cirrhosis (LC) is expected to become more common owing to its efficacy on portal hemodynamics. In this report we describe an alarming case of group B streptococcus (GBS) infection after splenectomy in a patient with LC.METHODS: A 72-year-old woman with a history of LC was admitted to our emergency department because of respiratory failure. The patient had received left lateral segmentectomy of the liver and splenectomy three months before admission. Pulmonary examination revealed significant wheezing during inspiration and expiration, but no crackles and stridor. Chest radiography and CT showed no infiltrates. A presumptive diagnosis of bronchial asthma caused by upper respiratory infection was made. Four days after admission, GBS infection was confirmed by blood culture and penicillin G was administered. Antibiotics were given intravenously for a total of 12 days.RESULTS: The patient was discharged on the 12th day after admission.CONCLUSIONS: Although efficacy of splenectomy in patients with LC has been reported, immune status should be evaluated for a longer period. Patients who have undergone splenectomy are highly susceptible to bacteria; moreover, LC itself is an independent risk factor for mortality in patients with sepsis. Since prophylaxis against GBS has not been established, immediate action should be taken. Emergency physicians should be aware of invasive GBS infection in the context of the critical risk factors related to splenectomy and LC, particularly the expected increase of splenectomy performed in LC patients.     

Plasma beta-endorphin levels and childhood intussusception

To determine whether childhood intestinal intussusception is associated with elevated plasma beta-endorphin levels, a series of patients was studied prospectively. Fourteen patients (age range between 3 months and 7 years) presented to two university pediatric emergency departments in Chicago with clinical symptoms and signs of intussusception. Venous blood (2cc) was withdrawn for plasma beta-endorphin determination, followed by barium enema. Plasma beta-endorphin levels were measured by radioimmunoassay. The mean beta-endorphin level of the 8 patients with barium enema proven intussusception was 14.1 ± 12.0 pg/ml. Two of these patients presented with marked lethargy and had beta-endorphin levels of 7.5 and 21.2 pg/ml. The mean plasma beta-endorphin level of the 5 patients with negative barium enema studies was 18.1 ± 10.0 pg/ml (P = 0.56). A sixth control patient had a plasma beta-endorphin level of 1569 pg/ml. In conclusion, childhood intestinal intussusception is not associated with elevated plasma beta-endorphin levels.     

主动脉粥样硬化性溃疡的16层CT诊断

目的：探讨应用16层螺旋CT诊断主动脉粥样硬化性溃疡的价值。方法：分析15例经16层螺旋CT诊断为主动脉粥样硬化性溃疡，14例以急性主动脉综合征就诊，1例以慢性病史就诊。观察溃疡的大小、形态、深度、数量及周围结构关系等。结果：单发溃疡12例，多发溃疡3例；14例急性患者均伴发范围较广泛的主动脉壁内血肿，其中10例合并中量以上血性胸腔、纵隔及心包积液，而1例慢性患者仅为局限的假性动脉瘤，不合并壁内血肿及血性积液等。结论：16层螺旋CT能够对主动脉粥样硬化性溃疡作出准确诊断，可作为急诊首选检查方法。     

Solitary rectal ulcer syndrome: A radiologic diagnosis?

The solitary rectal ulcer syndrome (SRUS) is an uncommon condition in which a solitary area of discrete ulceration is typically found on the anterior wall of the rectum. Between 1981 and 1983, we collected 8 pathologically proven cases of SRUS in which barium enema examinations had been performed (7 double-contrast, 1 single-contrast). Seven patients had rectal bleeding. On the original x-ray report, 4 cases were thought to be normal, but the pathologic tissue had been removed endoscopically in 2 of these cases prior to the radiologic study. The other 4 cases were thought to be abnormal, although the specific diagnosis of SRUS was not suggested in any case. In a blinded rereading of these 8 cases randomly interspersed with 29 other non-SRUS cases, however, the films were interpreted in light of recent radiologic experience with this condition. The same 4 cases were still thought to be normal. In the remaining 4 cases, barium enemas revealed thickened, edematous valves of Houston (3 cases) and a submucosal mass adjacent to the anal verge (1 case). The diagnosis of SRUS was suggested in all 4 cases with only 1 false-positive diagnosis due to a rectal stricture in a patient with endometriosis. Although barium enemas may be normal in patients with SRUS, the presence of thickened, edematous valves of Houston, particularly in a young patient with rectal bleeding, should suggest this condition.     

Radiology in primary small bowel adenocarcinoma

In a retrospective study, films from radiologic examinations of 13 patients with primary adenocarcinoma of the small bowel were reviewed. All patients had a barium examination and in 5 cases angiography was also performed. The barium studies disclosed strictures with overhanging edges in 11 patients, in 4 of whom there was a pronounced prestenotic dilation. Angiographically the predominant findings were in all cases hypovascularity and displacement of vessels. In 4 patients there was also encasement. Neovascularity was present in 2 patients. One patient showed angiographic changes due to intussusception.     

Refractory immune thrombocytopenia. Successful treatment with repeated cyclosporine A: two case reports

Treatment of chronic, severe refractory immune thrombocytopenia after splenectomy is difficult. Only less data exist on clinical use of cyclosporine A (CyA) in the management of refractory ITP. In this report, we describe two cases in which standard immunosuppressive therapy, other immunosuppression including cyclosporine A or splenectomy had no therapeutic effect. Even after splenectomy, recommended procedures were inefficient and critical thrombocytes count persisted. After repeated administration of cyclosporine A which had been ineffective prior to splenectomy; however, both patients achieved long‐term complete remission of the ITP. Side effects of CyA were moderate. The presented cases have confirmed the potential therapeutic effect of CyA in refractory post‐SE ITP.     

Spontaneous intramural oesophageal perforation

Spontaneous intramural oesophageal perforation appears at any level, but preferably on the posterior wall, and is usually longitudinal. Unlike the Mallory Weiss and the Boerhaave syndromes, females are more frequently affected. It appears with sudden retrosternal pain radiating to the epigastrium, neck and back, followed by haematemesis of small quantity and dysphagia. Vomiting is rare. In contrast to complete rupture, neither pneumomediastinum nor emphysema is observed, barium swallow being the diagnostic test of choice whenever this pathology is suspected. However, when the symptoms are not typical, endoscopy is a useful method for diagnosis. Treatment must be conservative, while surgery is suggested in the case of recurrent symptoms or big intramural haematomas with a high risk of perforation. A 74-year-old woman is presented. Endoscopy was performed as an emergency in suspected food impaction in the oesophagus. This case was diagnosed as spontaneous intramural oesophageal perforation. The patient also presented with oesophageal diverticulum and hiatal hernia. Conservative treatment was given, and the lesion cured.     

The penetrating aortic ulcer: pathologic manifestations, diagnosis, and management

The term "penetrating aortic ulcer" refers to an ulceration of an atheromatous plaque that extends deeply through the intima and into the aortic media. It may precipitate an intramedial dissection (usually localized) or may rupture into the adventitia to form a pseudoaneurysm. The typical patient with penetrating atheromatous aortic ulcer is elderly and has hypertension, atherosclerosis, and back or chest pain, but pulse deficit, stroke, aortic insufficiency, and compromise of a visceral vessel are not present. Classic aortic dissection and symptomatic thoracic aortic aneurysms are among possibilities in the differential diagnosis. Aortography demonstrates the presence of an aortic ulcer similar in appearance to gastric ulcers seen on barium examination; in addition, an intramural aortic hematoma may be present. Our experience with penetrating aortic ulcers in symptomatic patients indicates that conservative medical therapy leads to recurrence of symptoms and a need for surgical intervention. We present a case that illustrates the salient features of this distinct clinical entity.     

Relapse of idiopathic thrombocytopenic purpura caused by influenza A virus infection: a case report

We report a patient with idiopathic thrombocytopenic purpura (ITP) in remission, who relapsed as a result of an influenza A virus infection. A 41-year-old woman presented with fever elevation, coughing, and generalized petechiae. Her platelet count had decreased to 1 × 10⁹/l. She had been diagnosed with ITP at age 23, and continuous complete remission had followed steroid therapy and splenectomy. Influenza A antigen was positive in her pharyngeal aspirate, and oseltamivir was effective for her symptoms. Findings of a bone marrow smear were typical for ITP. Steroid therapy resulted in a second complete remission. Although the development of ITP caused by influenza infection and a relapse caused by an influenza vaccination have been previously described, a relapse caused by a sporadic infection has never been documented to our knowledge. Physicians should carefully monitor the hematological data of influenza patients, especially those with ITP, even in remission.     

Reduction of cecal volvulus by multiple barium enemas

It has been frequently stated that barium enema has no value in the treatment of cecal volvulus. We present a patient with cecal volvulus who was effectively treated by barium enema; however, multiple studies were required to achieve optimal results. In a subset of patients with cecal volvulus, laparotomy may be avoided by the use of barium enema.