Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022116 (ischemia)
 91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 25 years, from 1959 to 1984, esophageal substitution was performed in 32 patients. In most, the transverse colon was used, brought through the left chest on a vascular pedicle of the left colic artery. Indications for operation included: 21, esophageal atresia; 5, caustic injury; 3, peptic stricture; 2, esophageal varices with previous splenectomy; and 1, cartilagenous hamartoma of the esophagus. Six patients had failed prior reconstructions (1, gastric tube; 2, intrathoracic stomach; 1, presternal jejunum; 1, sloughed colon segment, 1, extensive stricture after primary repair). There was one postoperative death from fluid overload early in the series. Two patients had a localized leak at the upper anastomosis in the neck; neither resulted in stricture. One patient had a side leak in the lower intrathoracic colon, probably from an anchoring suture placed too deeply. Most patients had pyloroplasty with their operation. Four who did not required one later. Four patients required late reoperation for redundancy of the lower colon segment which emptied poorly; one lower colon was revised for stricture from exstrinsic compression at the substernal hiatus and another one for an inflammatory pseudopolyp with bleeding. There was no loss of a colon segment from ischemia. There is follow-up on all but one patient. Nineteen are more than ten years postoperative (mean of 18 years). Growth was assessed in that group. In atresia patients growth correlated with weight preoperatively and the presence or absence of associated anomalies. In the others growth was excellent in all but one patient. In our experience the colon conduit provides an excellent substitute esophagus for pediatric patients. The operation should have relatively low rate of major complications, most of which are avoidable, and most of which can be corrected to give a satisfactory long-term result.
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PMID:Colon interposition for esophagus in children. 408 10

II cases of major hepatic resections under total vascular isolation (TVA) are presented: 4 women and 7 men, age between 17 and 70 years (mean 39.6 years). In another 2 cases the method was abandoned because the patients did not tolerate the vena cava clamping. The main indication for TVA were large tumors located near the suprahepatic veins opening into the vena cava. The diagnosis in the 11 cases was: hepatocellular carcinoma--3 cases, cholangiocarcinoma--1 case, colo-rectal metastasis--1 case, hemangioma--3 cases, hamartoma--2 cases, diffuse suppuration of the right lobe--1 case. The warm ischemia time was between 25 and 50 min (mean: 36.8 min). There were no intraoperative complications. The mean quantity of transfused blood was 450 ml. Postoperatively two patients bled and were reoperated. Both subsequently developed liver failure and died and in both cases microscopy found histologic lesions of chronic hepatitis. The mortality was then 18.1%. Six patients (54.5%) developed postoperative complications. Worth noting are 2 cases of transient liver failure, both in patients with cancer. The ICU stay was between 2 and 14 days (mean 7.1) and the whole postoperative hospitalization was between 11 and 46 days (mean: 16).
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PMID:[Total vascular exclusion in liver surgery]. 901 63

Cystic mesenchymal hamartoma is an extremely rare, benign tumor. Rapid growth to a giant size can pose a threat not only in early childhood but also during fetal life. The experience with 2 antenatally diagnosed giant hepatic cysts with widely disparate approaches to management, treatment, and outcome is presented. A giant hepatic cyst was diagnosed on routine screening ultrasound scan. Because of its extremely massive size, the cyst was treated in utero with repeated aspirations, primarily for obstetric considerations. The infant did well, and the lesion was excised laparoscopically during the neonatal period. A second fetus with a giant hepatic cyst was not treated in utero, and the pregnancy continued to term. Nonimmune hydrops fetalis developed, and the fetus was delivered prematurely at 34 weeks. At birth, the infant was noted to have diffuse neurologic injury and no urine output despite normal-appearing kidneys. The lesion was excised during the neonatal period by open laparotomy. Observations at the time of surgery and pathologic studies of the placenta showed aneurysmal dilatation of the placental veins suggesting in utero compression of the fetal intraabdominal umbilical vein. The infant died shortly after birth. The experience with these 2 cases suggests the possibility that giant mesenchymal hamartoma diagnosed in utero may cause umbilical venous obstruction leading to ischemia during fetal life. Decompression of giant hepatic cysts may reverse this phenomenon and allow normal fetal development.
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PMID:Fetal therapy for giant hepatic cysts. 1237 77

A series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 53% of patients and dramatic improvement in the others. Surgical observations led us to propose a new anatomical classification according to the anatomical relationship between the hamartoma and the adjacent hypothalamus and third ventricle. Endoscopic disconnection seems to be a very safe way to treat hamartomas in intraventricular locations.
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PMID:Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification. 1262 81

Major ablative hepatic resection is often indicated in children with solid liver tumors, and reduction of operative blood transfusion is a primary goal. Total hepatic occlusion (THO) is an effective method that is well established in adults, yet its role in children is less well described. We describe our preliminary experience with THO in children assessing surgical outcome. The charts of seven children (ages 5 months to 7 years, weight 6-30 kg) who underwent THO during hepatectomy (four right and three left lobectomies) for liver tumors (hepatoblastoma in three, metastatic Wilm's tumor in two, mesenchymal hamartoma in one, and angiosarcoma in one) between January 1997 and June 2002 were reviewed. THO was established in all cases by clamping the supra- and infrahepatic inferior vena cava and the porta hepatis. Surgical parameters assessed included: 1) warm ischemia time, 2) operative blood transfusion, 3) operative complications, and 4) tumor resection margins. THO was successful in six of the seven cases (85.7%). In one case systemic hypotension unresponsive to fluid resuscitation developed at the outset with THO requiring conversion to pedicle clamping to perform the hepatectomy. Mean warm ischemia time during THO was 26 minutes (range 18-45 minutes). Mean estimated blood loss was 221 cm3 (range 50-800 cm3). Operative blood transfusion was required in one of six patients (15 cm3/kg). Excluding the "failed" THO case (intraoperative hypotension) there were no significant intraoperative or postoperative complications. All seven children had curative resections as indicated by "tumor-free" microscopic margins. We conclude that total hepatic occlusion can be performed safely and successfully for pediatric liver tumors. Operative blood transfusion appears to be minimized.
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PMID:Hepatectomy in children under total hepatic occlusion. 1285 17

The term Neuromuscular and Vascular Hamartoma (NMVH) was initially coined by Fernando and McGovern in 1982 in their report of 2 cases. Whether this lesion is truly hamartomatous or represents a "burnt-out" phase of varying chronic pathologies has been debated since that time. Examples of NMVH-like proliferations have been reported in the setting of diaphragm disease, Crohn's disease, radiation, and ischemia. Herein we present the case of a 73-year-old female with partial small bowel obstruction and a past surgical history significant for cholecystectomy and abdominal hysterectomy. A computed tomography scan revealed an ill-defined mass with the same density as muscle extending into the mesentery, worrisome for malignancy and generating the differential of lymphoma versus metastatic disease. Upon laparotomy, a 2.5 cm, constrictive, predominantly mural-based mass was identified. The more proximal bowel was dilated, and there were dense serosal adhesions. Grossly, the transmural lesion had a tan-yellow cobweb-like cut surface and the overlying mucosa was flattened. Histologically, the lesion contained fascicles of smooth muscle, irregularly spaced large nerve bundles, and thick-walled vasculature in a haphazard arrangement within a hypocellular fibroadipose stroma. No stigmata of Crohn's disease were observed, and the uninvolved enteric tissue was unremarkable. The patient's medical history was negative for chronic nonsteroidal anti-inflammatory use and was otherwise unremarkable. This case of an NMVH-like lesion is presented as a reminder of benign mass-forming lesions causing bowel obstruction and suggests that such lesions may develop secondary to a chronic inflammatory process.
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PMID:Neuromuscular and Vascular Hamartoma of the Small Intestine: An Exuberant Reparative Process Secondary to Chronic Inflammation. 2627 21

Neuromuscular and vascular hamartoma (NMVH) is an unusual lesion presenting as intestinal obstruction by stricture formation. It is characterized by a hamartomatous mass comprising haphazardly arranged mesenchymal tissue native to the intestinal mucosa and submucosa. We aimed to characterize the clinicohistopathological spectrum of NMVH in adult subjects with a search for an etiological clue. We reviewed 84 resected specimens (adult cases) of intestinal obstruction in our institute and diagnosed 4 cases with NMVH. A panel of special stains (Masson trichrome, Verhoeff-van-Gieson, and periodic acid-Schiff) and immunohistochemistry (smooth muscle actin, S100, Bcl2, CD34, vimentin, desmin, CD117, and CD3) were performed in all cases. All cases of NMVH showed characteristic hamartomatous mounds comprising haphazardly arranged smooth muscle, nerves, ganglia, vessels, and collagen with overlying mucosal ulceration. Adjacent bowel showed submucosal fibrosis, muscularis mucosae thickening, and duplication along with vasculopathy. A typical vasculopathy ("vessel-in-vessel" appearance) was seen in the submucosal and/or subserosal veins. Besides, different other forms of vasculopathic changes like obliterative venopathy and concentric myohypertrophy were also seen. One case had vasculitis and the patient died despite successful surgery. One other case was associated with lymphocytic ganglioneuronitis and granulomatous etiology. We conclude that NMVH can be multifactorial in origin although ischemia resulting from vasculopathy appears to be directly causative. The characteristic vasculopathy in the submucosal location may aid in the diagnosis of NMVH in small biopsy samples.
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PMID:Histopathologic Spectrum of Neuromuscular and Vascular Hamartoma With Special Reference to the Vasculopathic Phenomenon. 3176 Aug 40