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1.
BACKGROUND/PURPOSE: Bronchogenic cysts and esophageal duplications are usually considered as separate foregut malformations. Yet, both are thought to arise from the same embryological event, division of the embryonic foregut, and they share common histological characteristics, often making their clinical differentiation difficult. METHODS: A retrospective review of the cases of 68 children treated at a single institution between 1937 and 1995 was performed. Thirty children were girls (44%) and 38 were boys (56%). Ages ranged from newborn to 24 years. Complete records were available in all children. Fourteen of these 68 children were asymptomatic. RESULTS: Respiratory (54%) or gastrointestinal (13%) symptoms were the most frequent presenting problems. The majority of children were treated by resection of the cyst (52 of 68; 76%), while 9 of 68 (13%) required lobectomy for intraparenchymal lesions. Three children underwent marsupialization, with all of these children requiring additional surgery for recurrent disease. Five children (5 of 68; 7%) had multiple cysts. The mortality rate from this series was 10% (7 of 68). Two deaths were caused by perioperative exsanguination, one related to bleeding from a cyst lined with gastric mucosa with subsequent ulceration and hemorrhage into the esophagus. Two deaths occurred secondary to septic complications, one from an esophageal leak and the other from an intraparenchymal abscess. Two deaths were caused by respiratory failure; one was unrelated (SIDS). The majority of cysts found on histological review were lined by respiratory epithelium or bronchial glands (51 of 68; 75%). Gastrointestinal epithelium was present in cysts of nine children, only two of which were clinically diagnosed as esophageal duplications. Twenty-one cases (21 of 68; 31%) were classified as esophageal duplications based on the intramural location of the cyst, yet 15 of 21 (71%) contained respiratory epithelium, substantiating the hypothesis of the common origin of these lesions. CONCLUSIONS: The histological similarity and anatomic proximity of the "bronchogenic cysts" and the intramural "esophageal duplications" supports their common origin. The possible complications of bleeding, ulceration, infection, and obstruction of the esophagus or airway, should generally lead to prompt resection.  相似文献   

2.
Duplications of the gastrointestinal tract are congenital anomalies seen in about 0.2% of all children. These include the rare gastric duplications. Latter diagnosis is usually made in the first months after birth on recurrent vomiting by detection of an abdominal tumor. The most important imaging modality is ultrasonography. The case of a prematurely born child weighing 1900 g is presented in whom at the age of three weeks a gastric duplication of the greater curvature was diagnosed and who was successfully treated by resection. The postoperative follow-up for 24 months was uncomplicated.  相似文献   

3.
The histopathological, clinical, and radiological findings in 25 patients (median age 20.5 years; range 1.7-64.2 years) with gangliogliomas were assessed to correlate degree of astrocytic anaplasia and proliferative potential with recurrence or survival. Most patients (64%) presented with seizures (median Karnofsky Performance Score 90%; range 70-100%). Computerized tomography and magnetic resonance imaging showed nonspecific abnormalities. Neoplastic ganglion cells were defined as heterotopic, irregularly grouped, or having more than one nucleus of bizarre shape or size. The astrocytic component was moderately anaplastic in 15 cases and highly anaplastic (HAA) in 10. Eight patients had gross total resection, 11 had subtotal resection, and six underwent biopsy. Ten patients (five gross total resection, three subtotal resection, two biopsy) had no further treatment, 15 underwent external irradiation, and five had adjuvant chemotherapy. Twenty-four patients are alive 15-394 weeks (median 203.5 weeks) postoperatively; one with ganglioglioma-HAA died at 65 weeks. No tumor recurred after gross total resection. Duration of preoperative symptoms < 1 year, greater anaplasia, and age > 30 years at diagnosis may have increased the risk of recurrence after subtotal resection or biopsy by four, three, and two times, respectively (not significant). Bromodeoxyuridine labeling index (BUdR LI) was < 1% in eight non-recurring tumors and 1.3% in another recurring twice (second recurrence LI = 1.6%). Most patients with ganglioglioma have a good prognosis. After gross total resection, only observation is required. After subtotal resection or biopsy, recurrence is possible. BUdR labeling may guide further therapy.  相似文献   

4.
Gastric duplication cysts are rare in adults and usually asymptomatic. In most cases they are discovered incidentally by abdominal ultrasound, CT, or upper gastrointestinal x-rays. Most of the duplications (82%) are cystic and do not communicate with the stomach. Approximately half of the cases are associated with other congenital anomalies. We report a 59-year-old woman operated on for a pancreatic mass that proved to be a gastric duplication cyst. The cyst was resected and the postoperative course was uneventful.  相似文献   

5.
The genetic basis of sulfonamide resistance in six clinical isolates of Streptococcus pneumoniae was demonstrated to be 3- or 6-bp duplications within sulA, the chromosomal gene encoding dihydropteroate synthase. The duplications all result in repetition of one or two amino acids in the region from Arg58 to Tyr63, close to but distinct from the sul-d mutation, a duplication previously reported in a resistant laboratory strain (P. Lopez, M. Espinosa, B. Greenberg, and S. A. Lacks, J. Bacteriol. 169:4320-4326, 1987). Six sulfonamide-susceptible clinical isolates lacked such duplications. The role of the duplications in conferring sulfonamide resistance was confirmed by transforming 319- or 322-bp PCR fragments into the chromosome of a susceptible recipient. Two members of a clone of serotype 9V, one susceptible and one resistant to sulfonamide, which are highly related by other criteria, were shown to have sulA sequences that differ in 7.2% of nucleotides in addition to the duplication responsible for resistance. It is postulated that horizontal gene exchange has been involved in the acquisition (or loss) of resistance within this clone. However, five of the six resistant isolates have distinct duplications and other sequence polymorphisms, suggesting that resistance has arisen independently on many occasions.  相似文献   

6.
OBJECT: The indications, operative findings, and outcomes of vestibular schwannoma microsurgery are controversial when it is performed after stereotactic radiosurgery. To address these issues, the authors reviewed the experience at two academic medical centers. METHODS: During a 10-year interval, 452 patients with unilateral vestibular schwannomas underwent gamma knife radiosurgery. Thirteen patients (2.9%) underwent delayed microsurgery at a median of 27 months (range 7-72 months) after they had undergone radiosurgery. Six of the 13 patients had undergone one or more microsurgical procedures before they underwent radiosurgery. The indications for surgery were tumor enlargement with stable symptoms in five patients, tumor enlargement with new or increased symptoms in five patients, and increased symptoms without evidence of tumor growth in three patients. Gross-total resection was achieved in seven patients and near-gross-total resection in four patients. The surgery was described as more difficult than that typically performed for schwannoma in eight patients, no different in four patients, and easier in one patient. At the last follow-up evaluation, three patients had normal or near-normal facial function, three patients had moderate facial dysfunction, and seven had facial palsies. Three patients were incapable of caring for themselves, and one patient died of progression of a malignant triton tumor. CONCLUSIONS: Failed radiosurgery in cases of vestibular schwannoma was rare. No clear relationship was demonstrated between the use of radiosurgery and the subsequent ease or difficulty of delayed microsurgery. Because some patients have temporary enlargement of their tumor after radiosurgery, the need for surgical resection after radiosurgery should be reviewed with the neurosurgeon who performed the radiosurgery and should be delayed until sustained tumor growth is confirmed. A subtotal tumor resection should be considered for patients who require surgical resection of their tumor after vestibular schwannoma radiosurgery.  相似文献   

7.
BACKGROUND: Extrapancreatic, extraduodenal and extralymphatic (ectopic) gastrinomas have been reported only rarely. The frequency, locations, and surgical outcome of these lesions are unknown. METHODS: From 1982 to 1997, 215 patients with Zollinger-Ellison syndrome were evaluated prospectively at the National Institutes of Health and 142 patients (66%) underwent standardized surgical exploration and resection. Eight patients (5.6%) (six men and two women; mean age, 41 years) had primary gastrinoma located in ectopic sites. Long-term follow-up was derived from a prospective database. RESULTS: Ectopic gastrinoma tissue was identified and resected in the liver (three patients), common bile duct (one patient), jejunum (one patient), omentum (one patient), pylorus (one patient), and ovary (one patient). Seven patients (88%) were cured biochemically after resection and five patients (63%) have sustained cures, with a mean follow-up of 7.5 years (range, 0.4 to 11.7 years). One patient with a jejunal primary gastrinoma had a biochemical recurrence at 2 years, and another with a primary hepatic gastrinoma had a recurrence 6 years after resection. A patient with a pyloric primary gastrinoma was not cured. CONCLUSIONS: Extraduodenal, extrapancreatic, and extranodal gastrinomas are encountered in 5.6% of patients who undergo exploration with curative intent. If no gastrinoma is found in the usual locations, other ectopic sites should be examined carefully. Resection of these primary ectopic tumors can lead to durable biochemical cures.  相似文献   

8.
OBJECTIVE: The authors report on indications and results of local excision of tumors of the ampulla of Vater. SUMMARY BACKGROUND DATA: Local excision of ampullary tumors has been performed for nearly a century but remains controversial. The use of this procedure for benign conditions is clear, but its place, if any, in the management of ampullary malignancy is debated. METHODS: The presentation, evaluation, and treatment of 26 patients who underwent local resection of ampullary tumors between January 1987 and November 1994 are reviewed. RESULTS: There were 16 men and 10 women, with a median age of 58 years. Eighteen patients had adenomas, whereas 8 patients had adenocarcinomas. Patients presented predominantly with jaundice (50%), pain (35%), and pancreatitis (27%) and were evaluated with endoscopic retrograde cholangiopancreatography and biopsy. All patients with benign lesions had accurate preoperative biopsies. Two of eight patients shown intraoperatively to have malignant lesions had preoperative biopsies read as benign. There were no deaths. Postoperative complications included two wound infections and one episode each of cholangitis, lower gastrointestinal bleeding, and adhesive gastrointestinal obstruction. All patients had prompt resolution of jaundice if present before surgery, and the mean postoperative stay was 7.5 days. Six of eight patients with malignant lesions have had recurrent disease. CONCLUSIONS: Local excision of malignant ampullary tumors is effective palliative therapy when the patient is unfit for the Whipple procedure. Ampullary resection usually is curative for patients with benign lesions without a polyposis syndrome. In this series, intraoperative frozen section routinely was accurate.  相似文献   

9.
A retrospective study was performed between 1980 and 1995 on 38 recipients of proximal tibial allografts after wide resection of benign and malignant tumors. Twenty-one (55%) patients experienced one or more complications. Of the 26 patients who received chemotherapy, 15 (58%) experienced one or more complications, whereas of the 12 patients who did not receive chemotherapy, six (50%) experienced one or more complications. In the chemotherapy group, there were 12 (46%) fractures, four (15%) infections, three (12%) nonunions, and four (15%) instabilities. In the nonchemotherapy group there were three (25%) infections, two (17%) fractures, one (8%) instability, and one (8%) nonunion. These complications were managed adequately with multiple subsequent surgical procedures. Three patients underwent amputations for deep wound infections. Twelve (32%) patients underwent removal of the allograft, and the limb was salvaged by reallografting or by total knee arthroplasty. The results of both groups were 66% (25 of 38 patients) satisfactory (good or excellent). The chemotherapy group had a significantly higher incidence of fractures. All other complication rates and functional outcomes were not significantly different between these groups.  相似文献   

10.
We reviewed the records of 16 patients with true macrodactyly and analyzed the typical clinical features and methods of treatment. Fourteen feet were involved in 13 patients (one was bilaterally affected). Three hands were involved in three patients. Clinically, all lesions in the hands and lesions in 11 of 14 feet involved the preaxial side. There was multiple digit involvement in two hands and 11 feet. Progressive macrodactyly (10 feet and two hands) was more common than the static type (four feet and one hand). Proximal involvement of the sole or palm occurred in seven feet and one hand; all cases were of progressive macrodactyly. Enlargement of the metatarsals or the metacarpals was frequent (11 feet and two hands). The growth behavior and extent of bony involvement were similar in patients with hand involvement and those with foot involvement. Fourteen patients had additional clinodactyly, either medial or lateral. The toes of eight feet had angular deformities in the sagittal plane; most were angulated dorsally. Nine patients underwent surgery and two had repeated surgery. The reduction procedures included debulking, ray resection, toe resection, phalangeal resection, and phalangeal epiphysiodesis; the corrective procedures included wedge osteotomy, interdigitalization, and split thickness skin graft. Of the nine patients surgically treated, five had good results and four had fair results. Of the seven patients without surgical repair, three had fair results and four had poor results. Surgical debulking, phalangeal resection, ray resection, and phalangeal epiphysiodesis produced significant improvement in macrodactyly of the feet and hands. Toe resection was not as beneficial.  相似文献   

11.
Chromosome engineering in mice enables the construction of models of human chromosomal diseases and provides key reagents for genetic studies. To begin to define functional information for a small portion of chromosome 11, deficiencies, duplications, and inversions were constructed in embryonic stem cells with sizes ranging from 1 Mb to 22 cM. Two deficiencies and three duplications were established in the mouse germline. Mice with a 1-Mb duplication developed corneal hyperplasia and thymic tumors, while two different 3- to 4-cM deficiencies were embryonically lethal in heterozygous mice. A duplication corresponding to one of these two deficiencies was able to rescue its haplolethality.  相似文献   

12.
Paralogous genes from several families were found in four human chromosome regions (4p16, 5q33-35, 8p12-21, and 10q24-26), suggesting that their common ancestral region underwent several rounds of large-scale duplication. Searches in the EMBL databases, followed by phylogenetic analyses, showed that cognates (orthologs) of human duplicated genes can be found in other vertebrates, including bony fishes. In contrast, within each family, only one gene showing the same high degree of similarity with all the duplicated mammalian genes was found in nonvertebrates (echinoderms, insects, nematodes). This indicates that large-scale duplications occurred after the echinoderms/chordates split and before the bony vertebrate radiation. It has been suggested that two rounds of gene duplication occurred in the vertebrate lineage after the separation of Amphioxus and craniate (vertebrates + Myxini) ancestors. Before these duplications, the genes that have led to the families of paralogous genes in vertebrates must have been physically linked in the craniate ancestor. Linkage of some of these genes can be found in the Drosophila melanogaster and Caenorhabditis elegans genomes, suggesting that they were linked in the triploblast Metazoa ancestor.  相似文献   

13.
BACKGROUND: The aim of this study was to characterize patients with pulmonary metastasis of parathyroid carcinoma and to evaluate the long-term effect of surgical and medical therapy. METHODS: Seven patients with pulmonary metastasis of parathyroid carcinoma were treated between 1980 and 1992. Six patients underwent resection of pulmonary metastases, and one patient has had long-term bisphosphonate therapy alone. Bisphosphonate was also given before or after operation to three patients. RESULTS: Two patients underwent a unilateral thoracotomy for a single pulmonary lesion, and four other patients with multiple lesions underwent staged bilateral thoracotomies. The postoperative serum calcium level returned to normal after each thoracotomy in three patients who were alive and well 3, 8, and 12 years after the first thoracotomy. Hypercalcemia persisted in the other three patients. In two of the patients, bisphosphonate therapy was also unable to control hypercalcemia. In one patient the serum calcium level has been maintained in the 13 mg/dl range by bimonthly bisphosphonate therapy alone for 3 years. CONCLUSIONS: The aggressive surgical approach to pulmonary metastasis of parathyroid carcinoma was shown to be effective for palliation in selected patients. Bisphosphonate therapy is an alternative to resection but has only a temporary calcium-lowering effect.  相似文献   

14.
EA Bakalakos  JA Kim  DC Young  EW Martin 《Canadian Metallurgical Quarterly》1998,22(4):399-404; discussion 404-5
Hepatic resection remains the only potentially curative treatment for metastatic colorectal cancer. This retrospective review study was undertaken in an attempt to identify factors that influence patient survival following hepatic resection for metastatic colorectal cancer. From January 1978 to December 1993, a total of 301 patients underwent a total of 345 planned hepatic resections for metastatic colorectal cancer. Of those, 245 patients had one resection, 44 had two resections, and 12 had three resections. For all patients the overall median survival was 20.6 months, operative mortality was 1.1%, and overall morbidity was 17.2%. Average hospital stay was 9 days. Statistical analysis included univariate analysis using log rank comparisons, Kaplan-Meier survival curves, and multivariate analysis using Cox proportional hazards regression. The statistically significant factors that influenced survival were distribution of liver metastases, unilobar versus bilobar (p = 0.0001), resected versus nonresected (p < 0.0001), and tumor-free surgical margins versus positive margins (p = 0.001). Surprisingly, the disease-free interval and the original stage of the primary tumor did not predict survival (p = not significant). Other factors that had no influence on survival were type of resection, size and number of liver metastases, ABO blood group, and the number of perioperative blood transfusions. For those patients who underwent resection of unilobar metastases with tumor-free margins, the 5-year survival rate was 29% with a median survival of 35 months and eight survivors > 7 years. In addition, one patient with bilobar disease had survival > 7 years and five patients who had resection of hepatic metastases and extrahepatic cancer simultaneously had survival > 3 years. Our data support the concept that patients with unilobar metastatic disease who undergo surgical resection with tumor-free surgical margins can be afforded a significant opportunity at long-term survival with acceptable morbidity, mortality, and hospital stay. Also, certain patients with bilobar or extrahepatic disease (or both) who undergo complete resection can enjoy a long-term survival. In these subgroups of patients resection should be considered on an individual basis.  相似文献   

15.
We describe five cases of gastrointestinal leishmaniasis in patients with human immunodeficiency virus infection and review 10 additional cases reported in the literature. All of the patients had CD4+ cell counts of < 200/mm3, and AIDS had been previously diagnosed for 12 patients. Fever and splenomegaly were present in 46% of cases. Thirteen patients had digestive symptoms; these symptoms included diarrhea (6), dysphagia and/or odynophagia (6), abdominal pain (2), epigastric pain (2), gastrointestinal hemorrhage (1), and rectal discomfort (1). The regions of the digestive tract most frequently affected by Leishmania organisms were the duodenal mucosa (90%) and the gastric mucosa (75%). Endoscopy showed normal-appearing mucosa in 45% of cases. In 10 cases the diagnosis of visceral leishmaniasis was first made by biopsy of the gastrointestinal mucosa. In most cases treatment with antimonial agents was not effective.  相似文献   

16.
BACKGROUND: The aim of the present study was to review the authors' experience of the diagnosis and management of food bolus obstruction in a Chinese population. METHODS: During the period 1990-96, 31 patients were treated for food bolus obstruction. The records of these patients were retrospectively reviewed. RESULTS: Eighteen patients (58%) had a history of previous gastric surgery and 13 patients (42%) had an intact gastrointestinal tract. The most common site of impaction was the terminal ileum, and a synchronous site of food bolus obstruction was present in 16% of cases. A total of 91% of the food bolus retrieved was phytobezoar. In 15 cases, the food bolus was successfully milked into the caecum. Enterotomy for removal was necessary in 12 patients, and two underwent small-bowel resection for necrosis. CONCLUSION: Food bolus obstruction affects not only those who have had previous gastric surgery, but also a significant proportion of those with an intact gastrointestinal tract.  相似文献   

17.
The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.  相似文献   

18.
BACKGROUND: Fiberoptic flexible sigmoidoscopy (FFS) is routinely requested preoperatively as part of evaluation of pelvic masses to exclude colonic involvement by the tumor or concurrent colonic neoplasm. The aim of our study was to evaluate the utility of preoperative FFS in patients with suspected gynecologic malignancy. METHODS: FFS, performed using a 60 cm sigmoidoscope, evaluated (1) presence of bowel involvement by the tumor, (2) extrinsic compression by the tumor, and (3) presence of colonic neoplasms. FFS findings were correlated with surgical findings. RESULTS: A total of 107 women underwent preoperative FFS and subsequent surgery. Eleven patients (11%) had lower gastrointestinal symptoms. At surgery, 63% of pelvic tumors were malignant and 37% were benign. The most common abnormality at FFS was colonic polyps in 23 patients (21%). Colonic adenomas were found in 11 patients (10%). Extrinsic compression by the tumor without mucosal abnormalities was seen in 15 patients (14%). The most common intraoperative finding was tumor adhering to the bowel in 18 patients requiring dissection, but only 1 patient required bowel resection. Eight of these 18 patients had preoperative lower gastrointestinal symptoms. All 15 patients with extrinsic compression at FFS had tumor adhering to the bowel. CONCLUSIONS: Pelvic masses cause extrinsic compression at FFS in 14% of patients. This is suggestive of tumor adherent to the bowel at surgery. However, bowel resection is rarely required because of tumor involvement. Most patients with bowel adherence by tumor have lower gastrointestinal symptoms. Colonic adenomas are found in one tenth of patients, mostly in patients older than 50 years of age. Preoperative FFS does not change the surgical management of pelvic tumors. Screening FFS is indicated in all patients with pelvic tumors over age 50, as in persons with average risk, but is otherwise unnecessary in evaluation of pelvic masses.  相似文献   

19.
BACKGROUND/OBJECTIVE: Pelizaeus-Merzbacher disease (PMD), an X-linked recessive dysmyelination disorder, is caused by mutations in the proteolipid protein (PLP) gene. However, missense mutations were only found in a fraction of PMD patients, even in families that showed linkage with the PLP locus on Xq22. Here we describe the use of an extended protocol that includes screening for both missense mutations and duplications. METHOD: Two groups of patients were analyzed, one group with 10 independent PMD families and one group with 24 sporadic patients suspected of PMD. Missense mutations in the PLP gene were identified by sequencing. PLP gene duplications were detected by quantitative polymerase chain reaction and/or Southern blot analysis. RESULTS: Sequencing of the PLP gene revealed four mutations in group 1 and one mutation in group 2. However, inclusion of duplication analysis in the screening protocol raised the amount of mutations found in group 1 from 40 to 90%, and in group 2 from 4 to 25%. CONCLUSIONS: These results demonstrate that duplications of the PLP gene are the major cause of PMD. Furthermore, it appears that the phenotype resulting from PLP duplications is relatively mild, and that many probands are nontypical PMD patients.  相似文献   

20.
The records of 15 patients with metastatic carcinoma to skeletal muscle treated between 1979 and the present were reviewed. Fourteen patients were referred with a diagnosis of soft tissue sarcoma and one with suspected infection. There was a previous diagnosis of carcinoma in eight patients but seven patients had no prior diagnosis of a known malignancy. Primary tumors were lung (eight), melanoma (two), gastrointestinal (one), kidney (one), and bladder (one). No primary tumor could be identified in two patients. Local control of metastatic lesions was achieved by radiotherapy in 11 patients as an initial measure. Two patients underwent wide excision and one declined treatment for local tumor control. Eight patients died within 12 months of presentation and survival analysis indicated a 25% overall survival at 60 months. Two patients remained free of disease at 132 months and 72 months. From this study and a review of 52 cases reported in the literature, the authors are unable to find any clinical or radiographic characteristics that distinguish metastatic carcinoma to muscle from soft tissue sarcomas. Surgical resection can be reserved for cases in which radiation does not provide local control.  相似文献   

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