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Desmoid tumours exhibit fibroblastic proliferation and arise from fascial or musculoaponeurotic structures. Despite their benign microscopic appearance, and their negligible metastatic potential, the propensity of desmoid tumours for local infiltration is potentially significant in terms of deformity, morbidity and mortality due to pressure effects and obstruction of vital structures and organs. The rarity of desmoid tumours, coupled with the variability in their clinical course, renders these lesions a vexing entity, and makes demonstration of the efficacy of any specific intervention difficult. Failure to recognize the potential for malignant behaviour in this tumour renders desmoids susceptible to inadequate treatment. This distinct pathological entity is reviewed with a specific focus on aetiology and treatment., (Copyright Harcourt Publishers Limited.)

Background: The routine use of nasogastric tubes in patients undergoing elective abdominal operation is associated with an increased incidence of postoperative fever, atelectasis, and pneumonia. Previous studies have shown that nasogastric tubes have no significant effect on the incidence of gastroesophageal reflux or on lower esophageal sphincter pressure in healthy volunteers. We hypothesized that nasogastric intubation in patients undergoing laparotomy reduces lower esophageal sphincter pressure and promotes gastroesophageal reflux in the perioperative period., Methods: A prospective randomized case-control study was undertaken in which 15 consenting patients, admitted electively for bowel surgery, were randomized into 2 groups. Group 1 underwent nasogastric intubation after induction of anesthesia, and Group 2 did not. All patients had manometry and pH probes placed with the aid of endoscopic vision at the lower esophageal sphincter and distal esophagus, respectively. Nasogastric tubes, where present, were left on free drainage, and sphincter pressures and pH were recorded continuously during a 24-hour period. Data were analyzed with 1-way analysis of variance., Results: The mean number of reflux episodes (defined as pH < 4) in the nasogastric tube group was 137 compared with a median of 8 episodes in the group managed without nasogastric tubes (P =.006). The median duration of the longest episode of reflux was 132 minutes in Group 1 and 1 minute in Group 2 (P =.001). A mean of 13.3 episodes of reflux lasted longer than 5 minutes in Group 1, with pH less than 4 for 37.4% of the 24 hours. This was in contrast to Group 2 where a mean of 0.13 episodes lasted longer than 5 minutes (P =.001) and pH less than 4 for 0.2% of total time (P =.001). The mean lower esophageal sphincter pressures were lower in Group 1. CONCLUSIONS. These findings demonstrate that patients undergoing elective laparotomy with routine nasogastric tube placement have significant gastroesophageal reflux in the perioperative period and a reduced ability to clear refluxed acid from the distal esophagus. Due to the associated risk of postoperative pulmonary complications, we recommend that nasogastric intubation be performed on a selective rather than routine basis.

Background: The immunomodulatory effects of hypertonic saline (HTS) provide potential strategies to attenuate inappropriate inflammatory reactions. This study tested the hypothesis that administration of intratracheal aerosolized HTS modulates the development of lung injury in pancreatitis., Methods: Pancreatitis was induced in 24 male Sprague-Dawley rats by intraperitoneal injection of 20% L-arginine (500 mg/100 g body weight). At 24 and 48 h, intratracheal aerosolized HTS (7.5% NaCl, 0.5 mL) was administered to 8 rats, while a further 8 received 0.5 mL of aerosolized normal saline (NS). At 72 hours, pulmonary neutrophil infiltration (myeloperoxidase activity) and endothelial permeability (bronchoalveolar lavage and wet:dry weight ratios) were assessed. In addition, histological assessment of representative lung tissue was performed by a blinded assessor. In a separate experiment, polymorphonucleocytes (PMN) were isolated from human donors, and exposed to increments of HTS. Neutrophil transmigration across an endothelial cell layer, VEGF release, and apoptosis at 1, 6, 12, 18, and 24 h were assessed., Results: Histopathological lung injury scores were significantly reduced in the HTS group (4.78 +/- 1.43 vs. 8.64 +/- 0.86); p < 0.001). Pulmonary neutrophil sequestration (1.40 +/- 0.2) and increased endothelial permeability (6.77 +/- 1.14) were evident in the animals resuscitated with normal saline when compared with HTS (0.70 +/- 0.1 and 3.57 +/- 1.32), respectively; p < 0.04). HTS significantly reduced PMN transmigration (by 97.1, p = 0.002, and induced PMN apoptosis (p < 0.03). HTS did not impact significantly upon neutrophil VEGF release (p > 0.05)., Conclusions: Intratracheal aerosolized HTS attenuates the neutrophil-mediated pulmonary insult subsequent to pancreatitis. This may represent a novel therapeutic strategy.

Introduction: Ileal pouch-anal anastomosis (IPAA) remains the gold standard for patients with refractory ulcerative colitis. Pouchitis causes considerable morbidity in 40% of patients with IPAA. This study examined the role of increased epithelial apoptosis in the etiology of pouchitis., Methods: Following ethical approval pouch biopsies taken from patients with a history of pouchitis were compared with age-matched controls from patients who were pouchitis free. Apoptosis was detected immunohistochemically using a monoclonal antibody (M30) and terminal deoxyribonucleotidyl transferase (TDT)-mediated dUTP-digoxigenin end labeling (TUNEL). Villous atrophy was assessed histologically and correlated with levels of apoptosis. Epithelial Fas-ligand (L) was also assessed immunohistochemically., Results: A significant increase in TUNEL staining was seen at the epithelial but not at the lamina propria level for known pouchitis patients versus controls (0.091 vs 0.035; P < 0.01). Similarly, epithelial M30 immunoreactivity (0.225 vs 0.082; P < 0.05) and villous atrophy (0.035 vs 0.10; P < 0.05) were significantly increased in pouches with previous pouchitis when compared with normal pouches. Upregulation of Fas-L expression was characteristic of this epithelium. Mononuclear cells were strongly positive for Fas-L. Increased epithelial levels of apoptosis correlated with increased levels of villous atrophy., Conclusions: Our data suggest a role for elevated Fas-Fas-L (CD95-CD95L)-mediated epithelial apoptosis in the etiology of pouchitis. Increased levels of villous atrophy may result from increased apoptosis and thereby predispose to infection by otherwise apathogenic organisms., (Copyright 2001 Academic Press.)

Our aims were to determine the incidence of gallstones in a cohort of patients infected with hepatitis C for 20 years, and to analyse the outcome of all patients infected with hepatitis C undergoing laparoscopic cholecystectomy. A hepatitis C screening programme in place in Ireland since 1994 identified 965 patients with hepatitis C antibodies out of 62,667 patients screened. The hepatology unit of Cork University Hospital has 318 patients with hepatitis C. Of patients identified by screening, 201 were post partum women infected via contaminated Anti-D immunoglobulin administered in 1977. Thirty-five (17.4%) of two hundred and one patients with hepatitis C since 1977 had developed gallstones after twenty years. A total of 34 patients with hepatitis C underwent laparoscopic cholecystectomy. One patient required conversion to open cholecystectomy. There were no complications and no mortality. There was a low rate of cirrhosis (11%) on examining liver histology. The incidence of gallstones in a cohort of patients infected with hepatitis C for twenty years approximates to that of the general population. The low rate of cirrhosis in this group may be related to a low consumption of alcohol. Laparoscopic cholecystectomy is a safe procedure in patients with mild chronic liver disease caused by hepatitis C.

Background: Paediatric foreign body (FB) ingestion is a common problem and while most can be managed conservatively, a sub-population require intervention., Aims: To establish clear guidelines for management of paediatric FB ingestion., Methods: A retrospective chart review analysing all paediatric admissions with FB ingestion over a 10-year period from 1990 to 1999., Results: Of 339 patients presenting to the accident and emergency department with FB ingestion, 59 required admission. Ingestion was accidental in 93.0% of patients. The reasons for admission were as follows: large FBs; dangerous FBs; and living far from the hospital. Nineteen patients (32.2%) were discharged without intervention. Thirty-seven (62.7%) required endoscopic retrieval. In two, the FB was not identified at endoscopy. Only three (5%) required surgery., Conclusion: Conservative management of FB ingestion in the paediatric population is possible in the majority of cases. However, a minority require intervention. While guidelines for intervention are ill-defined, definitive indications include symptomatic patients, or dangerous objects.

Background: Neutrophils play a crucial role in host defense against infections, but their inappropriate infiltration and activation within tissues can cause host tissue damage through release of reactive oxygen metabolites, metalloproteinases, and proinflammatory cytokines. The termination of a neutrophil-mediated inflammatory response is effected through programmed cell death or apoptosis. Delayed neutrophil apoptosis is associated with proinflammatory diseases, such as the systemic inflammatory response syndrome. Surgery induces a profound inflammatory response; therefore, neutrophil apoptosis of patients undergoing elective surgery was investigated., Methods: Nonseptic patients undergoing elective orthopedic surgery while under epidural anesthesia had neutrophils and platelet-poor isolated from whole venous blood harvested at 4 time points: pre-epidural, 45 minutes postepidural but before surgical intervention, 1 hour postsurgical incision, and 24 hours postsurgery. Neutrophil apoptosis was quantified at 1, 12, and 24 hours in culture by immunofluorescence flow cytometry of annexin V and propidium iodide staining and confirmed by TUNEL (terminal deoxynucleotidyl transferase nick end labeling) assay for DNA strand breaks. Serum cytokines were quantified by specific enzyme-linked immunosorbent assay., Results: Spontaneous neutrophil apoptosis after elective surgery was significantly (P < .001) inhibited with an effect evident within an hour of surgical incision and persisting at 24 hours postsurgery. The addition of patients' 24 hour postoperative plasma to healthy neutrophils markedly (P < .01) reduced neutrophil apoptosis, whereas plasma taken an hour after surgical incision was ineffective. Interleukin (IL)-6 was notably increased (1395 +/- 196 pg/mL, P < .01) 24 hours postsurgery and at this postoperative concentration inhibited (P < .01) apoptosis of normal neutrophils. Levels of other inflammatory mediators (IL-1 beta, tumor necrosis factor alpha, granulocyte-macrophage colony-stimulating factor, soluble Fas, soluble Fas ligand) were unaltered. The anti-inflammatory cytokine IL-10 was only slightly increased 24 hours postsurgery (8.32 +/- 2.99 pg/mL); however, the addition of recombinant human IL-10 (10 ng/mL) counteracted (P < .05) inhibition of neutrophil apoptosis induced by IL-6 and post-surgery plasma., Conclusions: These results identify marked inhibition of neutrophil apoptosis after elective surgery and suggest that the inhibition of neutrophil apoptosis in the postoperative period is, at least in part, a result of soluble circulating factors. The marked imbalance favoring proinflammatory over anti-inflammatory cytokine release in the immediate postoperative period mediates the overwhelmingly antiapoptotic net capacity of postsurgery plasma.

In the normal resolution of an acute inflammatory response apoptosis of neutrophils is essential to maintain immune homeostasis and limit inappropriate host tissue damage by decreasing neutrophil tissue load, function, and release of phlogistic reactive oxygen species and proteases. The systemic inflammatory response syndrome (SIRS), a massive pro-inflammatory immune state, is associated with delayed neutrophil apoptosis, however, the systemic circulating factors and intracellular signal transduction pathways important in regulating neutrophil apoptosis in SIRS are poorly described. Neutrophils isolated from patients with SIRS on admission to the intensive care unit showed significantly (p<.01) delayed spontaneous neutrophil apoptosis compared with healthy neutrophils as quantified using annexin V-FITC and terminal deoxyuridine triphosphate (dUTD) nick end labeling (TUNEL) flow cytometry methods. Plasma from SIRS patients markedly (41.5+/-7.2%, p<.01) inhibited apoptosis of healthy neutrophils compared with controls (69.7+/-4.8%) indicating the presence of soluble circulating factors that can modify the expression of neutrophil apoptosis. Various pro-inflammatory (IL-6, granulocyte macrophage colony-simulating factor, interleukin (IL)-1beta, tumor necrosis factor-alpha) mediators, known to modulate neutrophil apoptosis in vitro, were elevated in the plasma of our cohort of SIRS patients compared with controls. However, the anti-apoptotic effect of SIRS plasma was specifically attenuated (75.5%, p<.01) by neutralizing SIRS plasma of granulocyte macrophage-colony-stimulating factor, but not IL-6, IL-1beta, tumor necrosis factor-alpha. Although the anti-inflammatory cytokine IL-10 was elevated in SIRS plasma (median level 7.2 pg/mL), further boosting SIRS plasma with recombinant human IL-10 (10 ng/mL, levels found in septic shock patients) significantly countered (63.8%, p<.01) the inhibitory effect of SIRS plasma on neutrophil apoptosis. Suppression of neutrophil apoptosis was concomitant with delayed spontaneous elevation of reactive oxygen species, quantified as peroxide production, and reversed by addition of neutralizing antibodies to GM-CSF, and recombinant human IL-10 to SIRS plasma. These results identify circulating GM-CSF as a significant inhibitor of neutrophil apoptosis in patients with SIRS, and that this effect can be countered by boosting SIRS plasma with IL-10. GM-CSF and IL-10 appear to modulate neutrophil apoptosis by altering reactive oxygen species generation in neutrophils.

Purpose: The aim of this article is to emphasize the increased risk of developing metachronous ovarian tumors after resection of rectal cancer., Method and Results: We report the case of a postmenopausal female patient who, five years after anterior resection, developed a primary ovarian malignancy that invaded a rectal anastomosis and in so doing mimicked a recurrence of a Dukes A rectal cancer. To our knowledge, such an occurrence has not been described previously in the literature., Conclusion: This case illustrates the possible benefits of routine prophylactic oophorectomy at the time of colorectal cancer resection.

Achalasia of the esophagus developed in two male siblings soon after birth, and they were successfully treated by surgery. Persistent signs resulted in the later diagnosis of Hirschsprung's disease. One required subtotal colectomy and ileoanal anastomosis, and the other is managing well on conservative treatment. Genetic analysis of the genes encoding the RET protooncogene, endothelin-3, and the endothelin-3 receptor did not show any defect. Familial achalasia of the esophagus in combination with Hirschsprung's disease has never been reported.

The motor nerve transplantation (MNT) technique is used to transfer an intact nerve into a denervated muscle by harvesting a neurovascular pedicle of muscle containing motor endplates from the motor endplate zone of a donor muscle and implanting it into a denervated muscle. Thirty-six adult New Zealand White rabbits underwent reinnervation of the left long peroneal (LP) muscle (fast twitch) with a motor nerve graft from the soleus muscle (slow twitch). The right LP muscle served as a control. Reinnervation was assessed using microstimulatory single-fiber electromyography (SFEMG), alterations in muscle fiber typing and grouping, and isometric response curves. Neurofilament antibody was used for axon staining. The neurofilament studies provided direct evidence of nerve growth from the motor nerve graft into the adjacent denervated muscle. Median motor endplate jitter was 13 microsec preoperatively, and 26 microsec at 2 months, 29.5 microsec at 4 months, and 14 microsec at 6 months postoperatively (p < 0.001). Isometric tetanic tension studies showed a progressive functional recovery in the reinnervated muscle over 6 months. There was no histological evidence of aberrant reinnervation from any source outside the nerve pedicle. Isometric twitch responses and adenosine triphosphatase studies confirmed the conversion of the reinnervated LP muscle to a slow-type muscle. Acetylcholinesterase studies confirmed the presence of functioning motor endplates beneath the insertion of the motor nerve graft. It is concluded that the MNT technique achieves motor reinnervation by growth of new nerve fibers across the pedicle graft into the recipient muscle.

During a four-year period, 308 patients presented following ingestion of foreign bodies. Ingestion was accidental in 272 cases (88.3%) and deliberate in the remainder. Symptoms at presentation included dysphagia, odynophagia, nausea and vomiting, chest pain and pharyngeal discomfort. Sixty-eight patients were asymptomatic. A policy of expectant management and selective endoscopy was employed. Following initial assessment 202 patients (65.6%) were discharged without treatment, 30 (9.7%) of whom were later reviewed as outpatients and did not require admission. Forty-nine patients (16%) were admitted for treatment; 27 had oesophagoscopy, five bronchoscopy and two had foreign body extraction with direct laryngoscopy. In nine patients who were endoscoped, no foreign body was identified. Twenty-seven others were referred to the otorhinolaryngology service in another hospital. There were no deaths in the group and morbidity was 1.2%. We conclude that a policy of selective endoscopy is safe and effective in the management of patients following ingestion of foreign bodies.

Deliberate and recurrent foreign body ingestion is a common problem among institutionalised patients. We review our experience with 36 cases of deliberate foreign body ingestion by prisoners or psychiatric patients, thirty of whom were institutionalised at the time of ingestion. Symptoms were frequently severe in the prison inmate group but, in contrast, psychiatric patients presented with few, if any, symptoms. A majority of objects pass spontaneously or remain in situ without complication. Twenty-four patients were discharged following initial evaluation and without specific treatment. Eight of these were reviewed electively and discharged within one week. Twelve patients were admitted for observation, seven of whom were discharged within 48 hrs. Upper gastrointestinal endoscopy was performed in four patients and an intragastric foreign body identified in two cases. Laparotomy was performed in two cases for unresolving mechanical intestinal obstruction. Management should be conservative when possible, with surgery indicated only for complications.

With the advent of laparoscopic cholecystectomy and the impending replacement of open common bile duct exploration, we have reviewed our ten year experience of conventional common bile duct exploration. Open cholecystectomy was performed in 1681 patients and the common bile duct was explored in 325 (19%). Following initial duct exploration, rigid choledo identified residual choledocholithiasis in 58 and periampullary neoplasia in 2 patients. Unexpected retained stones were identified in 3 patients (0.9%). In a further 4 patients, stones which could not be dislodged from the hepatic ducts were confirmed on 10 day T-tube cholangiogram--transduodenal sphincteroplasty had been performed at the time of original surgery in all four. The mortality was 1.9% overall and 1% during the last 5 years. Endoscopic exploration must compare favourably with these data for safe transition to less invasive techniques.

Sacral agenesis is a rare disorder of uncertain incidence that has been reported in diverse populations. Although usually sporadic and most commonly associated with maternal diabetes, there is a hereditary form which may occur in isolation or with a presacral mass (anterior meningocele and/or presacral teratoma) and anorectal abnormalities, which constitute the Currarino triad (MIM 176450). The radiological hallmark of hereditary sacral agenesis is a hemi-sacrum (sickle-shaped sacrum) with intact first sacral vertebra. Bowel obstruction is the usual neonatal presentation, but, unlike other neural tube defects, adult presentation is not uncommon. The major pathology is confined to the pelvic cavity and may present as a space-occupying lesion or meningitis due to ascending infection. All recurrences in families have been compatible with autosomal dominant inheritance except for those associated with the isomerism gene at Xq24-q27.1 (ref. 3). Several associated cytogenetic defects have been reported, including 7q deletions. Previous studies failed to detect linkage to HLA markers, but we now present evidence for a location on 7q36. The same region also contains a gene for holoprosencephaly, an early malformation of the extreme rostral end of the neural tube.

The increased risk of sepsis in patients following splenectomy has been well documented. Fear of overwhelming post-splenectomy sepsis (OPSI) has resulted in a generalized trend towards splenic salvage among surgeons. However, splenorrhaphy and attempts at splenic salvage may of themselves predispose to significant morbidity, sometimes more serious than increased susceptibility to infection associated with splenectomy. This study aims to assess the risk of splenectomy and subsequent asplenia. We reviewed 246 patients who underwent splenectomy over a 16 year period. Indications for splenectomy were considered under the following headings: haematological (N = 116), trauma (N = 69), visceral carcinoma (N = 28), incidental (N = 13) and miscellaneous (N = 20). There were 28 deaths in the series, primarily among those in the intra-abdominal carcinoma (13) and multiple trauma (13) groups. Two deaths were recorded among patients undergoing elective splenectomy for benign disease. Thrombo-embolic complications were recorded in nine patients; respiratory tract infection in 36 patients and intra-abdominal abscess in two patients. Two cases of post-splenectomy pneumococcal septicaemia were documented, neither of which was fatal. While not an entirely benign procedure, splenectomy can be performed relatively safely, especially when performed for benign disease in an adult population.

In an attempt to identify patients at risk of breast cancer, 9E 10 c-myc monoclonal antibody was used to detect the oncogene products in 64 breast tissue biopsies. There were 44 specimens of benign breast disorders: 21 from patients who developed subsequent breast cancer, and 23 from patients who did not. The remainder were malignant tumor biopsies from those who developed breast cancer. C-myc over-expression was identified in 12 (60%) of the breast carcinomas; it was also noted in 13 (62%) of the benign biopsies from patients who developed subsequent breast carcinoma. Expression was observed in only 3 (13%) controls--patients who did not develop cancer. A prospective study is now warranted to determine the clinical usefulness of our findings.

The rectoanal inhibitory reflex plays an important role in the normal mechanisms of anorectal continence. Anterior resection abolishes the reflex, but whether it recovers, particularly after inverted stapled anastomosis, is not clear. Anal manometry was performed on patients undergoing low anterior resection for carcinoma. Maximum anal resting pressure and the rectoanal inhibitory reflex were assessed preoperatively and up to two years postoperatively. The reflex was present in 43 of 46 patients (93 percent) preoperatively, in 8 of 45 patients (18 percent) on the 10th postoperative day, and in 6 of 29 patients (21 percent) between six months and one year following surgery. Twenty patients were studied more than two years postoperatively, and in 17 (85 percent) the reflex was demonstrated. In the majority of low anterior resection patients, the rectoanal inhibitory reflex is abolished by surgery, remains absent throughout the first year, and has recovered by the end of the second postoperative year. This may be important in the recovery of anorectal function in these patients.

Laparoscopic cholecystectomy is a reality and units must convert from open to endoscopic technique. To this end we have used the pig, whose biliary anatomy resembles the human, as a laboratory in vivo training model. Laparoscopic cholecystectomy has been performed in 35 animals. Techniques of pneumoperitoneum, vision, manipulation, clipping, ligation, cutting, diathermy and gall bladder removal were quickly mastered. All surviving animals underwent postmortem examination after 4 weeks. Following this laboratory programme, laparoscopic cholecystectomy in the human was introduced and has been completed successfully in 70 cases.

Marlex (polypropylene) mesh was used to replace the abdominal wall in massive incisional herniation in 50 patients. Seventeen unsuccessful attempts at primary repair had previously been made on nine patients. Patient follow-up ranged from 6 to 120 months (mean = 45 months). There was minor hernia recurrence in four (8 per cent) patients. In each instance it was due to partial detachment of the peripheral fixation of the patch. Complications were: wound infection, four (8 per cent); wound seroma, two (4 per cent); wound haematoma, one (2 per cent); and wound sinus, six (12 per cent). Complications did not necessitate removal of the patch in any case. Inadequate peripheral attachment of the patch has been the only cause of hernia recurrence and should be avoidable.

The technique of the Turnbull-Cutait pullthrough procedure as performed at the Cleveland Clinic for carcinoma of the rectum and other conditions is described. The results in 127 patients are reported. Eighty-four patients were operated upon for cancer of the rectum and 47 for miscellaneous benign conditions. The average distance of tumors from the anal margin was 7.6 cm. The average margin of resection was 4.1 cm. The overall operative mortality was 1.2% in the cancer group. Ischemic necrosis occurred in 1.2% of the cancer patients, and minor pelvic sepsis occurred in 7.1%. The five-year survival in Dukes' A, B and C carcinoma of the rectum was 100%, 57% and 53%, respectively. The incidence of pelvic recurrence of the tumor at 6% was within acceptable limits. The quality of bowel function following the pullthrough procedure is discussed. It is concluded that this pullthrough procedure has a significant role in the management of carcinoma of the rectum and other conditions.

The technique of pullthrough resection with delayed anastomosis for carcinoma of the rectum as performed at the Cleveland Clinic is described. A series of 84 cases of rectal cancer treated in this way is reported. The average tumour diameter was 4.5 cm, the average margin of resection was 4.1 cm and the average distance of the tumour from the anus was 7.6 cm. The incidence of necrosis of the pullthrough was 1.2 per cent. The overall 5-year survival was 63 per cent with 100 per cent, 57 per cent and 53 per cent for Dukes' A, B and C tumours respectively. The quality of bowel function following surgery is described in detail. It is felt that this procedure has an important place in sphincter conservation in carcinoma of the middle third of the rectum.