Jejunoileal atresia: a 27-year experience

PURPOSE: In this study, the authors review cases of jejunoileal atresia (JIA) to evaluate their surgical treatment strategy. METHODS: Eighty-eight neonates who underwent surgical repair for JIA were divided into four groups for the type of lesion: group 1, membranous (n = 23), group II, interrupted (n = 49), group III, multiple (n = 9), and group IV, apple-peel (n = 7). Group I patients were treated with membranectomy or bowel resection and anastomosis, group II with resection of the dilated bowel and one anastomosis, group III with two to six multiple anastomoses to preserve bowel length, and group IV with minimal bowel resection and bowel anastomosis. During surgery a uniform protocol was used to minimize bowel resection and to perform an end-to-end single layer anastomosis using either Halsted horizontal mattress or conventional interrupted sutures. Mortality, morbidity, days for functional recovery, and central venous nutrition (CVN) were included in the review. RESULTS: Of 88 patients, three died of causes unrelated to operation for JIA. Nine patients underwent an additional laparotomy for leakage (n = 4) and obstruction (n = 5). Oral feeding was allowed on day 5.4+/-4.3 and full caloric intake via the enteric route on day 12.5+/-10.0. Twenty-one patients required CVN for 32.4+/-19.1 days. None required a long-term treatment for the short bowel syndrome. CONCLUSION: This study concludes that efforts to preserve bowel length are laudable to avoid the short bowel syndrome and that an end-to-end single layer anastomosis contributes to early recovery of bowel function.     

The Waterston anastomosis with no deaths in the neonate

The Waterston anastomosis has been performed with no deaths in 11 consecutive neonates during the past 24 months. Seven neonates had pulmonary atresia with either single ventricle or hypoplastic right ventricle, 3 had tetralogy of Fallot, and one had severe pulmonary stenosis with a normal right ventricle. Six neonates were one day old and only one was older than 7 days. All have obtained symptomatic and documented (increase Po2) benefit. This level of success is attributed to (1) minimal delay between the recognition of cyanosis and operation (mean time between hospital admission and catheterization was 3 hours and between catheterization and operation, 4 hours); (2) correction of any base deficit prior to, during, and after operation; (3) accurate construction of the anastomosis to avoid excessive size, and (4) careful postoperative management of the pulmonary subsystem by experienced personnel. We have demonstrated that the high mortality rate previously reported for the Waterston anastomosis in the neonate can be markedly improved by an appropriate patient management program.     

A case of polyangiitis overlap syndrome

PURPOSE: To examine the quality of life after repair of esophageal atresia, follow-up studies were performed in 58 of 71 surviving patients (81.7%). METHODS: Fifty patients with primary anastomosis and all eight surviving patients with colon interposition were seen. The mean age was 25.3 years (range, 20 to 31). Symptoms were evaluated by a standardized interview. Quality of life assessment was performed using a visual analogue scale (0 to 100 points), the Spitzer Index (5 dimensions, 10 points), and the Gastrointestinal Quality of Life Index (GIQLI, 5 dimensions, 128 points). RESULTS: After primary anastomosis the estimated meal capacity was unrestricted in 46 patients (92%), but numerous symptoms such as recidivating cough (60%), hold up (48%), and short breath (30%) were reported. All symptoms except cough were seen more frequently in patients with colon interposition, and all of these patients suffered from periods of short breath. Quality of life scores were higher in patients with primary anastomosis compared with colon interposition. The difference in the visual analogue scale score did not reach statistical significance, but the mean Spitzer Index was 9.7 compared with 8.8 after colon interposition (P < .05). The GIQLI after primary anastomosis was similar to that in healthy controls and was significantly lower in patients with colon interposition. This was because of specific symptoms, which scored 49.3 after colon interposition compared with 61.7 after primary anastomosis (P < .05) and to 54.8 (SD 5) in healthy controls (P < .05). Physical and social functions, emotions, and inconvenience of a medical treatment scored similar in patients with primary anastomosis, colon interposition, and healthy volunteers. CONCLUSIONS: The long-term quality of life after primary anastomosis was excellent. Patients with colon interposition suffer more frequently from various gastrointestinal and respiratory symptoms, but they lead an otherwise normal life.     

Long-term morbidity following jejunoileal bypass. The continuing potential need for surgical reversal

OBJECTIVE: To review the late sequelae of jejunoileal bypass (JIB) and the potential role of late surgical reversal in ameliorating morbidity and mortality following JIB. DESIGN: Patients who underwent JIB between 1965 and 1977 were contacted and pertinent health-event information was gathered. Early sequelae were defined as disorders occurring within the first 2 years after JIB; late sequelae were those occurring after 2 years. Health events occurring between 0 and 23 years after JIB were documented. SETTING: A private, tertiary referral center. PATIENTS: Patients underwent JIB for morbid obesity that had failed medical and/or psychiatric interventions. MAIN OUTCOME MEASURES: Body mass index (BMI) (weight kilograms divided by the square of the height in meters), diarrhea, electrolyte imbalance, acute, and chronic liver disease, renal disease, JIB reversal, reason for JIB reversal, death, and cause of death. RESULTS: A total of 453 morbidity obese patients underwent JIB. By 2 years following JIB, the mean (+/- SD) BMI dropped from 49.3 +/- 8.1 to 31.1 +/- 0.8 and remained at this level until year 15, after which weight gradually increased (BMI, 35.4 +/- 3.1). The most severe early complication was acute liver failure, which occurred in 7% of patients and caused seven deaths. At 15 years, the actuarial probability of the most common serious late complications related to JIB were renal disease (37%), with two deaths; diarrhea (29%); and liver disease (10%), with three deaths. One hundred thirty-eight patients (31%) had a bypass reversal. The most common indications for reversal were diarrhea and electrolyte disturbance (29%), renal disease (19%), and liver disease (17%). Fifty-six patients died more than 30 days after JIB: 64% before JIB reversal, 13% at the time of reversal, and 23% subsequently. CONCLUSIONS: Jejunoileal bypass is associated with progressive accrual of serious, sometimes life-threatening complications. Lifelong follow-up for early diagnosis and surgical reversal before life is threatened should reduce the morbidity and mortality associated with this procedure.     

The role of a bidirectional cavopulmonary anastomosis in the correction and palliation of complex congenital cardiopathies

Between May 1990 and January 1998, 68 patients underwent bidirectional cavo-pulmonary anastomosis. We evaluated all patients in whom the bidirectional cavo-pulmonary anastomosis was associated with additional pulmonary flow (group A) and those in whom it was associated with biventricular repair (group B). Group A included 23 patients (33.8%), 14 males and 9 females, mean age 25 years and 6 months (range 4 months-16 years). Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). Group B included 13 patients (19.1%), 6 males and 7 females, mean age 13 years and 7 months (range 1 year-37 years). Diagnoses were pulmonary atresia with intact ventricular septum (4), Ebstein's anomaly (3), tetralogy of Fallot (3), atrioventricular canal (1), hypoplastic right ventricle (1), and pulmonary and tricuspid insufficiency (1). Four patients (17.3%) in group A died in the postoperative period, whereas there was no postoperative mortality in group B. Follow-up data were available in 31 patients (19 from group A, 13 from group B). Mean follow-up was 1 year and 6 months (range 30 days to 6 years). Evaluation was done by NYHA class functional status. In group A, 14 patients are doing well (NYHA I or II), while five patients (26.3%) underwent Fontan operation with one death. All group B patients are currently doing well (NYHA class I or II). In group A, complications were pericardial effusion (7), transient superior vena cava syndrome (5), pleural effusion (4), chylothorax (1) and rhythm disturbance (1). Complications in group B involved neurological events (2), pleural effusion (1) and rhythm disturbance (1). Bidirectional cavo-pulmonary anastomosis can be associated with additional pulmonary flow with good short- and intermediate-term outcome. Concern remains for the ability to properly regulate the amount of effective pulmonary blood flow. Bidirectional cavo-pulmonary anastomosis can be associated with biventricular repair in patients with diminutive right ventricles, amenable to anatomic complete correction, with good clinical outcome.     

Chloroquine versus pyrimethamine/sulphadoxine in the treatment of uncomplicated P. falciparum malaria in northern Kenya

Of 36 neonates with meconium ileus secondary to cystic fibrosis treated over a 10-year period, twenty-one (58%) had simple uncomplicated disease while fifteen (42%) had complications which included perforation (5), volvulus (6) and atresia (5). Gastrografin enema was employed in 20 infants with relief of obstruction in 8 (40%). Operative procedures consisted of resection and primary anastomosis in seventeen patients, stomas were fashioned in six, three had an enterotomy with irrigation only and two had Bishop-Koop enterostomy. Post-operative complications developed in 5 (18%) of these 28 patients. The overall survival rate was 97%. The one death occurred in an infant with short bowel syndrome, patent ductus arteriosus, hydrocephalus and pulmonary damage. There were eight additional patients who had meconium obstruction in the absence of cystic fibrosis.     

The Edmonton experience with venovenous extracorporeal membrane oxygenation

BACKGROUND/PURPOSE: Despite the proven effectiveness of venovenous extracorporeal membrane oxygenation (VV ECMO) in the treatment of neonates with severe respiratory failure, this technique is not widely used. The purpose of this study was to assess the authors' policy of preferred use of VV ECMO with a cephalad catheter and to compare the results with those of the Extracorporeal Life Support Organization (ELSO) Registry. METHODS: Charts of neonatal ECMO candidates were reviewed retrospectively. Data were collected for gestational age, birth weight, and diagnosis. Severity of illness was assessed by oxygenation index, lactate levels, and inotropic requirements before cannulation. Patients were divided into three groups: venovenous (VV), venoarterial (VA), and VV to VA ECMO. A cephalad catheter was inserted in the distal part of the jugular vein. RESULTS: Sixty-five neonates were supported with ECMO. Cannulation with a double lumen venovenous (VVDL) catheter was attempted in 63 neonates and successfully accomplished in 57. A survival rate of 86% was observed in neonates initially placed on VV ECMO. Five neonates initially placed on VV ECMO underwent conversion to VA ECMO. CONCLUSIONS: This study showed that the authors' preferred policy of VV ECMO did not result in an increase in mortality rate based on a comparison with ELSO data. VV ECMO with a cephalad catheter provides adequate support for unstable neonates with respiratory failure.     

Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia

BACKGROUND: Ebstein's anomaly, due to failure of delamination of one or more leaflets of the tricuspid valve (TV), is associated with varying degrees of tricuspid regurgitation (TR) and dysplasia of the right ventricle (RV). Although refinement of tricuspid valvuloplasty and plication techniques have opened the way to a satisfactory outlook for the majority of older children and adults, Ebstein's anomaly presenting at neonatal age, secondary to ineffective forward flow into the pulmonary and systemic circulation, has a reported mortality rate of as high as 75%. In order to improve the dismal outcome in neonatal Ebstein's anomaly, we have strived for early univentricular palliation. PATIENTS AND METHODS: Univentricular repair was performed in five neonates (median age 5 days; range 2-14 days) with Ebstein's anomaly, ductal dependent pulmonary blood flow, severe TR, absence of forward flow across the pulmonary valve, and small left ventricular (LV) area due to right-to-left bowing of the ventricular septum and ineffective LV loading (median indexed LV area 10.5 cm2/m2). In addition, two neonates had moderate pulmonary regurgitation (PR), one with additional pulmonary stenosis. In all patients, the indexed area of the combined right atrium and atrialized RV was greater than that of the combined functional RV, left atrium, and left ventricle (median 22.0 and 20.8 cm2/m2, respectively). The median preoperative systemic oxygen tension was 35 mmHg and the median pH 7.28. Repair consisted of TV closure with a pericardial patch (with the coronary sinus draining into the RV) (n = 3) or, in the presence of PR, resection of the dysplastic TV and division and oversewing of the main pulmonary artery (n = 2), as well as excision of the atrial septum, resection of redundant right atrial wall, and construction of an aortopulmonary shunt (n = 5). RESULTS: The median indexed LV area increased from 10.5 to 18.8 cm2/m2 as a result of more effective loading of the left ventricle. There was no intraoperative or late mortality. The patients were extubated at a median of 7 days postoperatively. At discharge, the median systemic oxygen tension was 46 mmHg. In all five patients, at 6, 7, 10, 12 and 16 weeks of age, a bidirectional cavopulmonary anastomosis has been constructed. CONCLUSIONS: In neonates with Ebstein's anomaly and ductal dependent pulmonary blood flow, rational palliation consists of the surgical creation of tricuspid atresia or, in the additional presence of PR or pulmonary stenosis, the creation of pulmonary atresia. These procedures may result in effective LV decompression and more effective volume loading of the left ventricle with increase of systemic output and improved clinical outcome.     

Liver transplantation for decompensated cirrhosis after jejunoileal bypass: a strategy for management

BACKGROUND: Although jejunoileal bypass results in end-stage liver disease in up to 100% of patients, little is known about outcome after liver transplantation. METHODS: The clinical courses of six patients who underwent liver transplantation at UCLA for decompensated cirrhosis owing to a jejunoileal bypass were reviewed. Liver function, allograft pathology, renal function, and nutritional status were assessed. RESULTS: Of the four patients with an intact jejunoileal bypass, two of the three who were biopsied had recurrent steatotic liver disease. The two patients whose jejunoileal bypass was reversed at the time of liver transplantation had lower alkaline phosphatase, lower creatinine, higher albumin, and higher cholesterol, and were more obese than their counterparts with intact bypasses. CONCLUSIONS: Patients undergoing liver transplantation for jejunoileal bypass-associated liver disease should, if possible, have their bypass reversed at the time of transplantation; otherwise, they must be followed closely and be biopsied routinely. Recurrent liver disease should prompt reversal of the jejunoileal bypass.     

Helicobacter pylori infection in patients with chronic pancreatitis and duodenal ulcer

BACKGROUND: The prevalence of duodenal ulcer is high in patients with chronic pancreatitis. Patients with simple duodenal ulcer without chronic pancreatitis are mostly Helicobacter pylori-infected, and the prevalence of IgG seropositivity is > 95%. The prevalence of H. pylori infection in patients with chronic pancreatitis is not known. METHODS: IgG antibodies against H. pylori were measured in a cross-sectional survey of consecutive patients who had their exocrine pancreas function examined with a Lundh meal test in the period 1988-95 and in a control group of patients with simple duodenal ulcer. RESULTS: Twenty-seven per cent of the patients with chronic pancreatitis had duodenal ulcer during the observation period. The prevalence of IgG antibodies against H. pylori was 22% in patients with chronic pancreatitis without duodenal ulcer as compared with 27% with non-organic abdominal pain. The prevalence of IgG antibodies against H. pylori was 60% in patients with chronic pancreatitis complicated by duodenal ulcer as compared with 86% in controls with simple duodenal ulcer. CONCLUSIONS: H. pylori infection contributes but may not be the only cause of duodenal ulcer in patients with chronic pancreatitis.     

Purification and characterization of a cytotoxin from Enterobacter cloacae

OBJECTIVE: To document our evolving surgical management of colonoscopic perforation and examine factors crucial to the improvement of patient care. DESIGN: We conducted a computer-based retrospective analysis of medical records (1980 through 1995). MATERIAL AND METHODS: Among 57,028 colonoscopic procedures performed, 43 patients (0.075%, or 1 perforation in 1,333 procedures) had a colonic perforation. Two additional patients were treated after colonoscopy performed elsewhere. The outcomes analyzed included surgical morbidity and mortality. RESULTS: Twenty-six women and 19 men who ranged in age from 28 to 85 years (median, 69) were treated for colonic perforation. More than 80% of perforations occurred during the latter half of the study period because of the increased volume of colonoscopic procedures (8 perforations among 12,581 examinations from 1980 through 1987 versus 35 perforations among 44,447 colonoscopies from 1988 through 1995). Emergency laparotomy was performed in 42 patients (93%). Perforations occurred throughout the colon: right side = 10; transverse = 9; and left side = 23. Three patients without evidence of peritoneal irritation fared well with nonoperative management. Most patients underwent primary repair or limited resection in conjunction with end-to-end anastomosis. In 14 patients (33%), an ostomy was created. One patient underwent laparotomy without further treatment. Intra-abdominal contamination ranged from none (31%) to local soiling (48%) to diffusely feculent (21%). Postoperative complications occurred in 12 patients and were associated with older age (P = 0.01), large perforations (P = 0.03), and prior hospitalization (P = 0.04). No postoperative deaths occurred. CONCLUSION: Despite a consistently low risk of colonic perforation, the increasing use of colonoscopy in our practice has resulted in an increased number of iatrogenic colonic perforations. In order to minimize morbidity and mortality, prompt operative intervention is the best strategy in most patients. Non-operative management is warranted in carefully selected patients without peritoneal irritation.     

Meconium ileus and intestinal atresia in fetuses and neonates

A collaborative study was performed to determine the different types and mechanisms of intestinal abnormalities during gestation. Cases had to fulfill one or more of the following three criteria: (1) meconium ileus, (2) intestinal stenosis or atresia, and (3) meconium peritonitis. Esophageal atresia, anorectal atresia, and abdominal wall defects were excluded. One hundred two cases were reviewed from the autopsies of 42 induced abortions, 22 stillborns, and the surgical findings in 38 neonates. Meconium ileus was detected mainly during the second trimester (28/38), and was associated with cystic fibrosis (15), fetal blood deglutition (4), infection (6), or multiple-abnormalities (10), in which three chromosomal aberrations were found. Intestinal stenosis or atresia was more commonly detected during the third trimester of gestation (46/56). Sixteen of the 30 duodenal malformations were associated with trisomy 21, whereas in the 26 small intestinal atresias, signs of distress or ischemia were most frequently detected. Only 8 of 25 meconium peritonitis cases were isolated. A total of 20 cystic fibrosis cases could be proved. In this series, functional abnormalities were observed predominantly in the second trimester and associated mainly with cystic fibrosis or amniotic fluid abnormalities. Anatomic lesions were commonly detected later on and associated with ischemic conditions, chromosomal aberrations, and even cystic fibrosis.     

Primary nonampullary/periampullary adenocarcinoma of the duodenum

Primary duodenal adenocarcinoma not involving the ampullary region is rare. Our aim was to review the outcome of these patients and determine the factors that affect survival. We performed a retrospective review of all patients with primary, nonampullary duodenal adenocarcinoma at the Cleveland Clinic Foundation from January 1986 through December 1996. Twenty-six patients with primary, nonampullary duodenal malignancies were identified. There were 16 adenocarcinomas, 3 gastrinomas, 3 stromal tumors, 3 leiomyosarcomas, and 1 carcinoid tumor. Patients with adenocarcinoma had symptoms present an average of 6.1 months. Tumors were identified by upper gastrointestinal contrast study and esophagogastroduodenoscopy in 90 per cent and 87 per cent of patients, respectively. Twelve of 13 (93%) cancers found in the third or fourth portion of the duodenum were adenocarcinomas. Seven of the 16 adenocarcinomas were resectable on exploration. Those that were contained within the serosa have not recurred (mean, 6 years); one of the two patients with locally invasive adenocarcinoma remains disease free. The average survival for patients with unresectable disease was 6.7 months. The 5-year survival rates were: all adenocarcinoma, 38 per cent; resectable, 86 per cent; and unresectable, 0 per cent. All patients presenting with weight loss or obstructive symptoms died of disease; those with melena survived long term. Patients with tumors other than adenocarcinoma had a 90 per cent 5-year survival. We conclude that patients typically present with a long history of symptoms. Distal duodenal malignancies are most frequently adenocarcinomas. Upper gastrointestinal contrast study or endoscopy is often diagnostic. Patients with weight loss and/or obstructive symptoms had invasive disease and a morbid prognosis. Aggressive surgery is warranted, and most with resectable disease (86%) had long-term survival.     

Membranous duodenal stenosis

The experience of our 16 patients treated for membranous duodenal stenosis is reported. Their treatment and course was analysed in a retrospective study. Eight patients were operated on within the first 16 days of life and in the remaining group surgery was performed at 1 month to 4 y of age. The presenting symptom leading to diagnosis was, in all but one case, non-bile-stained vomiting. Associated malformations were found in all but four patients, mostly morbus Down. The operative procedure performed was a partial excision of the duodenal membrane and a duodenoplasty in 10 patients, a duodenojejunostomy in 5 patients, and a duodenoplasty only in 1 patient. The postoperative course was without lethal complications. One late stricture in an anastomosis occurred. We conclude that in its presentation, duodenal stenosis differs from duodenal atresia, and can often be misinterpreted, resulting in a late diagnosis, and should be reported as a separate entity.     

Magnetic resonance cholangiography for evaluation of cholestatic jaundice in neonates and infants

Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes of cholestatic jaundice in neonates and infants. Nine anicteric infants (control group) aged 10 to 224 days (mean +/- SD, 8 +/- 65 days) and 15 neonates and infants with cholestatic jaundice, aged 22 to 142 days (mean +/- SD, 71 +/- 37) underwent MR cholangiography. The final diagnosis of extrabiliary atresia (6 patients) was based on laparotomy findings (4 patients) or autopsy (2 patients), while neonatal hepatitis (9 patients) was diagnosed according to the liver biopsy findings and clinical recovery during follow-up. Percutaneous liver biopsies were performed in all 15 patients. Results showed that the gall bladder and common bile duct (CBD) could be visualized using MR cholangiography in all patients in the control group. Nonvisualization of the CBD (6/6 patients) and demonstration of a small gall bladder (6/6 patients) characterized MR cholangiography findings in patients with biliary atresia. MR cholangiography failed to depict the CBD in one infant with hepatitis. We conclude that demonstration of the CBD by MR cholangiography in neonates and infants with cholestasis can be used to exclude the diagnosis of biliary atresia. In patients with cholestatic jaundice considered for exploratory laparotomy, preoperative MR cholangiography is recommended to avoid unnecessary surgery.     

Functional defects of Cx26 resulting from a heterozygous missense mutation in a family with dominant deaf-mutism and palmoplantar keratoderma

BACKGROUND: There is controversy about the impact on morbidity from delayed diagnoses of blunt hollow viscus injuries. A recent study suggested that the increased morbidity was primarily from delayed diagnosis of blunt duodenal injury (BDI). STUDY DESIGN: We studied the medical records from a 10-year period from June 1987 to June 1997 examining the data on 22,163 cases of blunt trauma. We assessed the incidence and consequences of delayed diagnoses of BDI, and identified preoperative factors associated with these delayed diagnoses. RESULTS: Thirty-five patients (0.2%) were identified in the retrospective study of the records from 22,163 blunt trauma patients to have sustained BDI. Of these, 25 patients (71%) were male. Ages ranged from 1 to 58 years (mean 18.8 years), and the predominant mechanism was motor vehicle accident in 18 patients (51%). Seven patients (20%) (group I) had a diagnostic delay of > 6 hours; 28 patients (80%) (group II) were diagnosed in < 6 hours. Six of the seven group I patients (86%) were evaluated initially with CT scans, and five (83%) showed findings suggestive of BDI. Among the 28 group II patients, 14 (50%) underwent initial diagnostic peritoneal lavage (DPL), and 14 (50%) had a CT scan. In seven of the group II patients (50%) who were initially evaluated by CT scan, there were findings suggestive of BDI. Diagnostic peritoneal lavage was initially equivocal (red blood cell count=5,000 to 100,000) in the remaining one group I patient compared with three of the group II patients who had DPL. Deterioration found on physical examinations prompted followup CT scans in 6 group I patients (86%), and the scans were diagnostic for BDI in all cases. CONCLUSIONS: Blunt duodenal injury is an uncommon entity. Despite the presence of suggestive CT and DPL findings, the diagnosis was delayed in 20% of the 35 patients whose records were examined in the study; this delayed diagnosis was associated with increased abdominal complications. Patients with persistent abdominal complaints and equivocal CT or DPL findings should undergo laparotomy or repeat CT scan evaluations.     

Comparative assessment of Fontan operation in modifications of atriopulmonary and total cavopulmonary anastomoses

OBJECTIVES: The optimal technique of the Fontan operation remains disputable. This investigation was aimed at the comparison of atriopulmonary and total cavopulmonary anastomoses. METHODS: The results of 81 operations of total cavopulmonary and 69 operations of atriopulmonary anastomosis, performed from 1983 to 1995 were analysed. A control study was carried out 1-10 (3.7 + 0.2) years after the operation in 80 patients (36 after total cavopulmonary and 44 after atriopulmonary anastomoses). 70 patients were studied several times. Cardiac catheterization was done in 70 patients. In 78 patients central hemodynamical indices were studied during exercise. RESULTS: Preoperative hemodynamical indices were not reliably different in the two groups. Early mortality after total cavopulmonary anastomosis was 15%, after atriopulmonary anastomosis 23%. The highest mortality was seen when the criteria of Choussat et al. (Choussat et al. Pediatric Cardiology. Edinburgh: 1977:559-566) were surpassed. In the early postoperative period after total cavopulmonary anastomosis circulatory insufficiency was less marked, transsudation from pleural cavities was reliably lower (15.3 + 1.2 versus 25.5 + 1.8 ml/kg/day, P < 0.01). Arrhythmias were more common after total cavopulmonary anastomosis (18.5 versus 12%). Late mortality after total cavopulmonary anastomosis was 6%, after atriopulmonary anastomosis 12%. 82% of patients after atriopulmonary anastomosis and 81% after total cavopulmonary anastomosis were in NYHA classes 1 and 2.7% of patients after total cavopulmonary anastomosis and 11% after atriopulmonary anastomosis were reoperated. Physical tolerance rose stepwise in both groups and by the third post-operative year reached 75% of normal level. At this time we saw the most optimal hemodynamical indices during exercise. However, the best hemodynamics during exercise were seen after atriopulmonary anastomosis. CONCLUSIONS: Total cavopulmonary anastomosis is accompanied by lower mortality rate and a more favourable course of early postoperative period. However, the best long-term functional results are obtained after atriopulmonary anastomosis.     

MPK38 expression is upregulated in immature T cells activated by concanavalin A

Between 1974 and 1994, 25 colonic interposition procedures were performed for esophageal replacement in 23 cases of esophageal atresia (EA) and 2 corrosive strictures. Nine patients had one-stage and 16 had two-stage reconstructions. The transthoracic route was used in 16 cases (64%) and the retrosternal route in 9 (36%). Average age at the time of operation in EA patients was 17 months (range 12-33), and the children with corrosive injuries were 3 and 6 years old. Mean age at follow-up was 11.8 years (2.3-20.5 years). There was no mortality in the series. One patient developed full-graft necrosis and had a gastric pull-up procedure later. One child had partial graft necrosis (3 cm at the cervical end), however, enough colon was available for reconstruction. Ten patients developed a leak from the cervical anastomosis (40%) and 7 developed a stricture at the cervical esophago-colonic anastomosis (28%). The strictures were treated by repeated esophageal dilatation, and 3 patients required revision of the anastomosis (12%). Other complications included acid reflux in 2 cases (8%), small-intestinal obstruction in 1 (4%), redundancy of the colon in 1 (4%), and chest infections in 2 (8%). Follow-up included assessment of the patient's symptoms, serial growth measurements (height and weight), and where relevant, endoscopy and a contrast swallow or meal. There was a 52% improvement in weight and height percentiles post-operatively. Each of the 19 patients who had barium swallows showed rapid transit and emptying without any significant delay or hold-up. Overall long-term results were excellent in 13 patients (52%), good in 7 (28%), and fair in 5 (20%). The colon conduit thus provides an excellent substitute esophagus in pediatric patients.     

Sternal wound complications--incidence, microbiology and risk factors

OBJECTIVE: Sternal wound complications, i.e. instability and/or infection (mediastinitis), are important causes of morbidity in patients undergoing cardiac surgery via median sternotomy. Coagulase negative staphylococci, a normal inhabitant of the skin, have evolved as a cause of sternal wound infections. Since these opportunistic pathogens often are multiresistant, they can cause therapeutic problems. METHODS: From 1980 through 1995 open heart surgery, was performed on 13,285 adult patients. Reoperation necessitated by sternal wound complications occurerd in 203 patients (1.5%). The incidence was 1.7% (168/9987) after coronary artery bypass grafting (CABG group) and 0.7% (35/3413) after heart valve surgery with or without concomitant CABG (HVR group). RESULTS: Factors independently related to sternal complications in the CABG group (variable odds ratio [95% C.I.]): year of surgery, 1.9 [1.3-2.8] in 1990-1992, 2.0 [1.4-2.9] in 1993-1995; female sex, 0.4 [0.2-0.6]; diabetic disease, 1.8 [1.2-2.5]; bilateral ITA procedure, 3.3 [1.1-7.7]; and postoperative dialysis, 3.1 [1.4-6.9]. In the HVR group they were: use of ITA graft, 3.7 [1.7-7.7]; early re-exploration because of bleeding 3.0 [1.1-8.2]; and postoperative dialysis 3.1, [1.4-9.3]. Multivariate models were used to compute the risk for sternal complications in each patient. However, the prognostic models based on these risk scores provided low sensitivity and low predictive value. Patients with sternal wound complications showed no increased early mortality but worse long-term survival even after adjustment for other factors (relative hazard in CABG group 1.9 [1.2-2.8]; in HVR group 2.1 [1.1-4.3]. CONCLUSIONS: The use of ITA grafts seems to be one of the most important factors related to sternal wound complications. However, patients at truly increased risk for this complication could not be identified on the basis of the risk factors considered in this study.     

Incidence of neoplastic polyps in the ileal pouch of patients with familial adenomatous polyposis after restorative proctocolectomy

PURPOSE: Although adenomatous polyps and even adenocarcinomas have been found in the terminal ileum of patients with familial adenomatous polyposis, the prevalence of neoplastic changes in the pouches of patients who have undergone restorative proctocolectomy is unknown. The objective of this study was to determine the frequency of pelvic pouch neoplasia in familial adenomatous polyposis patients after restorative proctocolectomy. METHODS: Patients in a polyposis registry who had undergone restorative proctocolectomy were recruited. Demographic, surgical, pathologic, and endoscopic data were obtained from patient records. Video pouchoscopy was done after two enemas and representative biopsies were taken. RESULTS: Of 102 eligible patients, 26 (17 males and 9 females) participated. Median age at ileal pouch-anal anastomosis was 31 (range, 12-58) years. Median follow-up period was 66 (11-156) months. Adenomas were found in the pouch of 11 (42 percent) patients, in the terminal ileum above the pouch in 1 patient, and in the anal canal of 4 patients. Among patients with pouch polyps, three patients had one lesion, three patients had two lesions, and five patients had more than ten lesions. The incidence of polyps increased steadily with time from restorative proctocolectomy. There was no relation between the incidence of pouch polyposis and the severity of colonic or duodenal disease. CONCLUSIONS: Proctocolectomy and ileal pouch-anal anastomosis is associated with a significant risk of pouch neoplasia in familial adenomatous polyposis patients. The severity of pouch adenomas was not related either to the severity of colonic or duodenal disease. The pelvic pouches of all patients with familial adenomatous polyposis who have undergone restorative proctocolectomy should be examined periodically.