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At least 847 records · Page 47Linked to original sources
PubMed2005

A Japanese family with Alport syndrome associated with esophageal leiomyomatosis: genetic analysis of COL4A5 to COL4A6 and immunostaining for type IV collagen subtypes.

BACKGROUND: In some families, X-linked Alport syndrome (AS) is associated with diffuse leiomyomatosis. We describe clinical, pathologic and molecular-genetic findings in a Japanese family with this inheritance mode of AS in association with leiomyomatosis. PATIENT: AS was diagnosed in a one-year-old boy with recurrent aspiration pneumonia caused by esophageal stenosis from leiomyomatosis. Diagnosis was confirmed by electron microscopy coupled with type IV collagen chain subtype staining in a renal biopsy specimen. His mother, who exhibited esophageal leiomyomatosis and is heterozygous for AS, showed a discontinuous staining pattern for collagen alpha5(IV) chain along the epidermal basement membrane in a skin biopsy specimen. Genetic analysis in the boy revealed the deletion of the first two exons of COL4A6 together with deletion of the 5' end of COL4A5. Despite administration of cyclosporin A, massive proteinuria has persisted in the boy, although renal function otherwise remains normal. CONCLUSION: Identification of an AS patient during infancy is extremely rare. Clinical manifestations, including macroscopic hematuria, cataracts and leiomyomatosis caused by the large deletion involving COL4A5 to COL4A6, led to early presentation with AS.

Collagen Type IV↗
PubMed1983

Esophageal cancer associated with primary early gastric cancer.

We treated 114 Japanese patients with primary esophageal cancer during a period between 1965 and 1981; and 5 (4.4 per cent) were associated with simultaneous primary gastric cancer. Four of the 5 patients were treated surgically and all of the coexisting gastric cancers were in the early stage. The presence of gastric lesions was correctly diagnosed preoperatively in two of four patients. Surgical reconstruction was done to prepare a new route for food ingestion using a segment of ileo-ascending colon. In the other two patients, gastric tube was prepared, because gastric cancers were detected by chance in the resected specimens in these patients. Based on our experiences, we wish to stress the importance of detailed preoperative gastric examination and of careful intraoperative inspection of the gastric mucosa in patients with esophageal cancer whose preoperative gastric examination provides inconclusive evidence due to the presence of esophageal stenosis.

Adenocarcinoma↗
PubMedDate not supplied

[Endoscopic sclerotherapy of esophageal varices with 80% alcohol. Experience with 46 cases].

We are presenting the results obtained by endoscopic sclerotherapy with 80% alcohol in fifty-five patients with upper GI bleeding caused by esophageal varices, with or without active bleeding at the moment of the endoscopic study. We obtained control of bleeding in 83.64% with a recurrence of bleeding of 16.36% during the first 15 days. At the same period of time we obtained a 16.36% mortality rate. In the forty six patients that concluded sclerotherapy, decrease in size of the varices occurred in 89.79% and eradication was obtained in 6.11% of the patients. We observed three types of complications: esophageal ulcers in 100% of the cases which healed in a period of three weeks. Retrosternal pain of short duration, in 45.65%, this pain appears when the sclerosant substance is injected. Esophageal stenosis in 5.45% which were treated with dilatations. Our conclusion is that 80% alcohol is an effective and safe sclerosant substance in the management of acute bleeding of esophageal varices and in the prevention of the immediate recurrence, but has the disadvantage of a great number of local complications.

Adolescent↗
PubMed1989

Mediastinal mass and radiolucent esophageal foreign body.

The diagnosis of radiolucent esophageal foreign bodies can be difficult, particularly in patients with predominant respiratory symptoms. The consequences of the impaction of a foreign body in the esophagus are serious, and esophageal stenosis, perforation, acquired tracheoesophageal fistulas are among the complications already reported. An unusual complication of a nondiagnosed radiolucent plastic coin that remained impacted for 11 months on the posterior esophageal wall of a 20-month-old child, who presented only with respiratory symptoms, is reported. The foreign body eroded through the esophageal wall, causing an intramural abscess that was initially interpreted as a mediastinal mass, and the patient was operated on with the diagnosis of a foregut duplication. Literature on this situation was reviewed, and the problems associated with the diagnosis and treatment of children with radiolucent esophageal foreign bodies are discussed.

Abscess↗
PubMed1992

Sugiura procedure in the surgical treatment of bleeding esophageal varices in children: long-term results.

Recurrent bleeding, the high occurrence of encephalopathy, and the impairment of hepatic function in the successful cases of portasystemic shunts have led to increasing dissatisfaction with these procedures in recent years. Between March 1974 and November 1990 we have operated on 15 children for bleeding esophageal varices using the Sugiura procedure (esophageal transection with paraesophagogastric devascularization). In two cases the entire procedure was performed via the thoracic approach. The spleen was left in place in five cases. We have had no mortality. Operative complications included bleeding in the early postoperative period in two children and partial leakage from the esophageal suture in two others. Follow-up was from 4 years 3 months to 16 years 8 months with an average of 10 years 4 months. Long-term results have been gratifying in 12 patients (80%) with disappearance of the varices and no evidence of recurrent bleeding. Three children (20% of the patients) had recurrent bleeding 4 1/2 years, 4 years 2 months, and 2 1/2 years after the surgical procedure. In all cases there was no evidence of esophageal stenosis, gastroesophageal reflux, or hiatal hernia, nor signs of encephalopathy nor impairment of hepatic function. In our opinion the Sugiura procedure is a valid procedure in the surgical treatment of esophageal varices bleeding in the pediatric age with a high rate of success (80%) and without late complications.

Child↗
PubMed2001

Crohn's disease of the esophagus: Three cases and a literature review.

Three cases of esophageal Crohn's disease (CD) are described, each with dysphagia and/or odynophagia caused by esophageal ulceration. All three patients had associated ileocolitis. One patient followed for a prolonged period responded to treatment with sulfasalazine and prednisone. A computer search back to 1967 produced 72 additional cases of esophageal CD. Among these 75 patients (total), who were, on average, 34 years old, esophageal disease was the presenting disease symptom in 41 patients (55%). The diagnosis was difficult in 13 patients, in whom no distal bowel disease was detected at the time of initial esophageal presentation. The most common presentation was dysphagia associated with aphthous or deeper ulcerations (52 patients). In 11 of these patients, oral aphthous ulcerations were also present. Esophageal stenosis or fistulas to surrounding structures were present in 27 patients and led to surgery in 17 patients. Most of the unfavourable outcomes were in this group of 27 patients with esophageal complications, including five deaths. Fourteen additional patients required surgery for CD of other areas. Responses of uncomplicated ulcerative disease of the esophagus tended to be favourable if the medical regimen included prednisone. Clinical patterns of esophageal CD were divided into three categories: ulcerative, stenosing and asymptomatic (acute disease in children).

Adrenal Cortex Hormones↗
PubMedDate not supplied

[Achalasia: report of two cases].

The first case, a two-month-old female infant, had frequent vomiting since 5 days of age. Dilated esophagus with narrowed distal end was noted on barium meal study. Panendoscopic examination revealed dilated esophagus and a pin-head sized, stenotic lumen at 15 cm from the incisor. Under the impression of esophageal stenosis, gastrostomy was performed for feeding. Esophagogram made at 6 months of age demonstrated good patency of the esophagus without stenosis and gastrostomy was subsequently closed at the age of 1 year. Unfortunately, swallowing disturbance had bothered her intermittently since. Barium esophagogram and panendoscopy made 8 years later again revealed distal esophageal obstruction with dilated proximal esophagus. The second case was a two and half year old boy who suffered from frequent regurgitation of undigested food for 6 months. Dilated esophagus with narrowing of the lower end of esophagus was noted on barium meal study. After admission, panendoscopic examination revealed the esophagus was very narrow at 25 cm from incisor and the proximal esophagus was dilated. The esophageal dilatation with mercury-weighted bougies was performed intermittently. At present he is doing well, and a barium meal study showed adequate esophageal emptying.

Barium Sulfate↗
PubMed2002

[Treatment policy for children with gastroesophageal reflux complicated by Barrett esophagus].

Barret's esophagus (BE) is a rare disease in children. It is caused by gastroesophageal reflux (GER). From 1996 to 1999 seventy-eighth children with GER were treated. Twenty-four-hour pH-metry and manometry of the esophagus, scintigraphy and contrast roentgenoscopy of the esophagus were used for diagnosis of GER. All the children underwent biopsy of mucosa membrane of distal esophagus. Morphologic examinations revealed BE in 16 (20.5%) children. Metaplasia of esophageal epithelium by intestinal type (IT) in combination with one by gastric type (GT) were revealed in 8 children, metaplasia by gastric type alone (epithelium of gastric and fundal parts of the stomach)--in 8 children. Six children with IT metaplasia of the esophagus with long strictures underwent extirpation of the esophagus with one-stage esophagoplasty. It esophageal stenosis is not long or is absent, fundoplication by Nissen (4 children) and drug therapy (6 children) are performed. It is concluded that in IT metaplasia of the esophagus with long peptic esophageal strictures resistant to bouginage extirpation of the esophagus with one-stage coloesophagoplasty is desirable. Other methods of treatment do not exclude probability of esophageal adenocarcinoma. These children should be followed up with esophageal biopsy each 6-12 months.

Adolescent↗
PubMed1992

[Characteristics of severe esophagitis in patients with collagen diseases].

Connective tissue disease may alter esophageal function generating symptoms due to gastroesophageal reflux and motor disturbances. Fifteen patients with connective tissue diseases and severe esophagitis defined by the presence of esophageal stenosis or ulcerations were studied. Diagnosis was made with radiologic, endoscopic and manometric studies. Dysphagia was present in 11 and gastroesophageal reflux in all. All patients has an hypotensive and shorter lower esophageal sphincter. Better therapeutic results were obtained with surgical treatment.

Adult↗
PubMed1991

Epidermolysis bullosa acquisita and associated symptomatic esophageal webs.

Epidermolysis bullosa acquisita (EBA) is a well-characterized, subepidermal blistering disorder associated with autoimmunity to type VII collagen, which is the collagen localized to anchoring fibrils within the dermoepidermal junction of skin. Although the full clinical spectrum of EBA is still being defined, it is known that the clinical features of EBA may be reminiscent of hereditary dystrophic epidermolysis bullosa, a scarring blistering disease of children that is commonly associated with esophageal stenosis. We describe a patient with EBA who had both an acral-predominant mechanobullous disease akin to dystrophic epidermolysis bullosa and an inflammatory, widespread bullous eruption reminiscent of bullous pemphigoid in association with esophageal webs and dysphagia. Although esophageal involvement is common in dystrophic epidermolysis bullosa, a review of the literature shows that this is the first bonafide case of EBA with symptomatic esophageal disease.

Aged↗
PubMed1994

Esophageal cancer treated with radiotherapy: impact of total treatment time and fractionation.

PURPOSE: Local control rate and survival rate of esophageal cancer treated with radical radiation therapy (RT) were analyzed with special respect to total treatment time and fractionation. METHODS AND MATERIALS: Between 1979 and 1992, 88 patients with Stages I-III esophageal cancer were treated radically with RT at Kyoto University Hospital and Wakayama Red Cross Hospital. Of the 88 patients, 52 patients were treated with conventional fractionation (1.7-2.0 Gy/day, five times/week), and the remaining 36 patients were treated with accelerated hyperfractionation (AHF). In 1989, we started AHF regimen for esophageal cancer. Daily fractionations were 2.0 Gy and 1.2 Gy (field-in-field), or 1.5 Gy and 1.5 Gy at 5- to 6-h interval. Most of the patients treated with AHF received the total radiation dose of 64-68 Gy. Twenty-seven patients were treated with intraluminal brachytherapy (IBT) as boost therapy following external RT. Fourteen patients were treated with IBT following AHF. RESULTS: The median of treatment time of AHF was approximately 2 weeks shorter than that of conventional fractionation. Local control rate at 1 year were 47% for AHF, which was significantly higher than that for conventional fractionation (22%, p < 0.05). The improvement of local control by AHF was responsible for a trend to an improved cause-specific survival (p = 0.07). Local control rates at 1 year were plotted as a function of total treatment time. The slope of the linear regression line was -2.3 +/- 0.5% per day (p < 0.025) for patients treated with external RT alone, indicating a 2.3% per day loss in local control. Pretreatment and treatment parameters were evaluated in a multivariate analysis for the end point of local control. T stage (T1, 2 vs. T3, 4; p = 0.003) and fractionation schedule (p = 0.03) were independent of prognostic significance. Patients could tolerate the AHF well, although esophageal stenosis was noted frequently as a late toxicity. CONCLUSION: Accelerated hyperfractionation was the most important treatment-related variable in this patient population. Total treatment time may have a significant impact on the treatment outcome for esophageal cancer.

Adenocarcinoma↗
PubMed1997

Enhanced effectiveness of photodynamic therapy with laser light fractionation in patients with esophageal cancer.

BACKGROUND AND STUDY AIMS: The fractionated application of laser light has been shown to enhance the effect of photodynamic therapy (PDT) on normal rat colon after photosensitization with 5-aminolevulinic acid. In a pilot study, we examined whether this modified laser treatment can also enhance the effect of PDT in patients with esophageal cancer after sensitization with hematoporphyrins. PATIENTS AND METHODS: Six patients (four cases of early esophageal carcinoma and two cases of advanced esophageal carcinoma, one of the latter patients having tumor overgrowth on a metal stent) were treated in nine sessions. In four sessions, laser energy was fractionated to enhance the PDT effect using a hematoporphyrin polyester. Irradiation was carried out 48 hours after sensitization. The total laser energy of 150 J/cm2 was applied either continuously or in fractions, with a single break of five minutes after 75 J/cm2. RESULTS: Among the three patients who underwent continuous laser light irradiation, one patient experienced a complete remission of two of three superficial esophageal cancers. The patient with tumor overgrowth on an implanted metal stent showed a partial response, with improvement of dysphagia without destruction of the stent, while in another patient with advanced esophageal cancer, the dysphagia did not improve after continuous laser treatment. Fractionated laser therapy led to complete remission in all three patients with early cancers, one of whom had failed to respond to previous treatment with continuous PDT. A partial remission was obtained in a fourth patient with a uT2 cancer who had also shown no remission after continuous PDT treatment. However, after fractionated laser therapy, three mild esophageal stenoses occurred, in comparison with none after continuous PDT. CONCLUSIONS: Light fractionation during PDT improved the effectiveness of the treatment in a small number of patients, and increased side effects such as the occurrence of mild esophageal stenosis. This modified treatment can be a promising approach in the effort to reduce the dose of hematoporphyrins or other sensitizers required, and to avoid prolonged skin sensitization or local stenosis.

Aged↗
PubMed1995

[Esophageal perforation by alkalis--a case report with conservative therapy].

In May 1993, a 59-year-old woman attempting suicide with toilet detergent (1% sodium hydroxide) was hospitalized as an emergency case. She developed sudden high fever (38-39 degrees C) on the 26th admission day. Thereafter diagnosis of left pyothorax due to perforation on caustic esophageal ulcer was made. Subsequent to thoracentesis continuous dranage of the left thoracic cavity ceased the fever after three days. Oral intake began on the 28th and extubation of the dranage was done on the 42nd after admission. Following hospital course of the patient was uneventful and the patient was discharged on the 111th day after admission. Esophagofluoroscopy taken at 6 months after discharge revealed no esophageal stenosis, and the patient returned to full social activities in good health.

Drainage↗
PubMed2004

Usefulness of EMR with an oblique aspiration mucosectomy device compared with strip biopsy in patients with superficial esophageal cancer.

BACKGROUND: EMR is now widely accepted as a treatment option for superficial esophageal cancer. Endoscopic oblique aspiration mucosectomy with an oblique aspiration mucosectomy device was performed, and the results were compared retrospectively with those of conventional strip biopsy. METHODS: Strip biopsy (April 1991 through October 1999) or endoscopic oblique aspiration mucosectomy (November 1999 through December 2002) was performed in a consecutive series of patients with superficial esophageal squamous-cell carcinoma. Variables assessed were size of resection specimens, rate of complete resection, and complications. RESULTS: Of the consecutive series of 66 patients with superficial esophageal cancer, 27 underwent strip biopsy and 39 had endoscopic oblique aspiration mucosectomy. The two groups were similar with respect to age, gender, and lesion macroscopic appearance. The endoscopic oblique aspiration mucosectomy group had a significantly greater mean depth of tumor invasion and had significantly more large lesions vs. the strip biopsy group. The mean longest diameter of resection specimens was significantly greater with endoscopic oblique aspiration mucosectomy (23.9 [5.2] mm) than with strip biopsy (15.2 [4.9] mm) (p<0.001). The en bloc resection rate was 33.3% (9/27) in the strip biopsy group and 46.2% (18/39) in the endoscopic oblique aspiration mucosectomy group (p=0.322). Despite larger lesion size in the endoscopic oblique aspiration mucosectomy group, the complete resection rate was similar between the strip biopsy (70.4%) and endoscopic oblique aspiration mucosectomy (74.4%) groups (p=0.783). The complete resection rate for lesions 30 mm or greater in diameter was slightly but not significantly higher in the endoscopic oblique aspiration mucosectomy group (84.4%) compared with the strip biopsy group (70.4%) (p>0.999). With respect to complications, the rates of bleeding and submucosal hematoma were similar. Esophageal stenosis occurred after the procedure in 3 patients in the endoscopic oblique aspiration mucosectomy group. All were managed by endoscopic dilation, and symptoms improved. CONCLUSIONS: Endoscopic oblique aspiration mucosectomy is safe and effective for the treatment of superficial esophageal cancer.

Aged↗
PubMed2002

Is endoscopic follow-up needed in pediatric patients who undergo surgery for GERD?

BACKGROUND: This study evaluated the role of endoscopy in the postoperative management of pediatric patients who undergo fundoplication for GERD. METHODS: Medical records of 109 otherwise healthy children who underwent operation for GERD from 1979 to 1996 were reviewed. Patients with respiratory symptoms or esophageal stenosis were excluded. All patients underwent endoscopic surveillance with endoscopy being performed in the early (within 1 year) and late (between 1 and 2 years) postoperative periods. Specifically evaluated were the appearance of the wrap and evidence of esophagitis. The risk of a recurrence of esophagitis based on wrap appearance and the presence of clinical symptoms in patients with endoscopic evidence of esophagitis were also evaluated. RESULTS: At early endoscopy 3 patients with an intact wrap and 8 with a defective wrap had esophagitis (not significant). At late endoscopy, 5 patients with an intact wrap and 17 with a defective wrap had esophagitis (p < 0.05). CONCLUSIONS: An intact wrap does not prevent recurrence of GERD. Such an occurrence is even more likely when endoscopy demonstrates a defective wrap. For all patients who have undergone fundoplication, endoscopic evaluation at 1 to 2 years is recommended to detect esophagitis in the absence of symptoms so treatment can be initiated before symptoms occur.

Adolescent↗
PubMed2006

External beam radiation therapy followed by high-dose-rate brachytherapy for inoperable superficial esophageal carcinoma.

PURPOSE: The aim of this study was to retrospectively evaluate the feasibility, efficacy, and tolerance of external beam radiotherapy followed by high-dose-rate brachytherapy in inoperable patients with superficial esophageal cancer. PATIENTS AND METHODS: From November 1992 to May 1999, 66 patients with superficial esophageal cancer were treated with exclusive radiotherapy. The median age was 60 years (range, 41-85). Fifty-three percent of them were ineligible for surgery owing to synchronous or previously treated head-and-neck cancer. Most of the patients (n = 49) were evaluated with endoscopic ultrasonography (EUS) or computed tomography (CT). The mean doses of external beam radiotherapy and high-dose rate brachytherapy were 57.1 Gy (+/-4.83) and 8.82 Gy (+/-3.98), respectively. The most frequently used regimen was 60 Gy followed by 7 Gy at 5 mm depth in two applications. RESULTS: Among patients evaluated with EUS or CT, the complete response rate was 98%. The 3-, 5-, and 7-year survival rates were 57.9%, 35.6%, and 26.6%, respectively. Median overall survival was 3.8 years. The 5-year relapse-free survival and cause-specific survival were 54.6% and 76.9%. The 5-year overall, relapse-free, and cause-specific survival of the whole population of 66 patients was 33%, 53%, and 77%, respectively. Local failure occurred in 15 of 66 patients; 6 were treated with brachytherapy. Severe late toxicity (mostly esophageal stenosis) rated according to the Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer scale occurred in 6 of 66 patients (9%). CONCLUSION: This well tolerated regimen may be a therapeutic alternative for inoperable patients with superficial esophageal cancer. Only a randomized study could be able to check the potential benefit of brachytherapy after external beam radiation in superficial esophageal cancer.

Adenocarcinoma↗