Late results of Senning operation.

OBJECTIVES: Few data exist for long-term results after the Senning operation for transposition of the great arteries. Sinus node dysfunction and systemic ventricular dysfunction have been the main problems. We evaluated risk factors for late death and the incidence of late death, sinus node dysfunction, and right ventricular dysfunction in 100 patients. METHODS: The study was a retrospective analysis with a mean follow-up time of 12.8 +/- 3.1 years. No patients were lost to follow-up. Patients were divided in 2 groups according to ventricular septal defect (73 simple, 27 complex). The electrocardiogram, ambulatory electrocardiogram, echocardiogram, and chest radiograph were reviewed for each patient. RESULTS: The overall mortality rate was 10%. The actuarial survival was 90% (simple) and 78% (complex); the probability of staying in sinus rhythm was 34% and 7%, and the probability of normal right ventricular function was 52% and 39%, respectively, 15 years after operation. The incidence of sinus node dysfunction increased gradually over time, although the incidence of right ventricular dysfunction increased rapidly after 10 years of follow-up. Late deaths, arrhythmias, and right ventricular dysfunction were significantly more frequent in the complex group. Right ventricular dysfunction and active arrhythmias were risk factors for late death. CONCLUSION: Long-term follow-up after the Senning operation shows increasing incidence of sinus node dysfunction and right ventricular dysfunction over time. Deteriorating right ventricular function is a major concern. Its early recognition and initiation of appropriate management to preserve cardiac function is an important follow-up goal.

Treatment with human chorionic gonadotrophin for cryptorchidism: clinical and histological effects.

The efficacy of hCG treatment was studied in 182 cryptorchid patients. The efficacy of the treatment correlated with the initial position of the testis. None of the abdominal testes reached a normal position, whereas 90% of high scrotal testes descended during the treatment. Fifty-four boys who were treated unsuccessfully with hCG and 29 untreated boys were biopsied. The biopsies were performed on 32 scrotal and 87 maldescended testes to examine the state of the organ and the effects of hCG treatment. In biopsies, the volume densities of seminiferous tubules, interstitial tissue and blood vessels were counted, and the sections screened for interstitial bleeding. Significant differences between scrotal and maldescended testes were found in all of the volume densities measured. Interstitial bleeding occurred rarely in scrotal testes, whereas in maldescended testes it was frequently apparent. hCG treatment induced a significant increase in the volume density of both interstitial tissue and blood vessels. Even though the hCG treatment induced measurable, possibly harmful, changes both in scrotal and maldescended testes, our data do not prove that hCG treatment causes permanent damage to the testis.

Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects.

Between 1948 and 1980, 107 of 164 patients survived after repair of a congenital diaphragmatic defect. Sixty of the survivors (mean age, 29.6 years; SD, 9.0 years) underwent clinical examination, chest radiography, spirometry, and diffusing capacity measurement 11 to 41 years after the diaphragmatic repair. Twenty-seven of the 60 had body plethysmography, xenon 133 radiospirometry, and a test of bronchial hyperreactivity. Subjective physical performance was below average for eight patients (13%), seven patients (12%) had asthma, and four patients (7%) reported increased susceptibility to respiratory infections. Thirty-one patients (52%) had ventilatory impairment, which was obstructive in nine (15%), restrictive in seven (12%), and obstructive and restrictive in 15 patients (25%). Nine (35%) of the 26 patients tested had bronchial hyperreactivity. The presence of ventilatory impairment and bronchial hyperreactivity correlated with the initial clinical severity of the affliction. Chest asymmetry (29 patients; 48%) and scoliosis (16 patients; 27%) were more common among patients with ventilatory impairment than among those with normal spirometric findings. Ventilatory impairment and thoracic deformities are common in adults with repaired diaphragmatic defects. Surveillance should begin in infancy and continue into adulthood.

Chest wall and spinal deformities in adults with congenital diaphragmatic defects.

Between 1948 and 1980, 107 of 164 patients survived after repair of congenital diaphragmatic defects. To study chest wall and spinal deformities among these patients, 60 survivors (mean age, 29.6 +/- 9.0 years) underwent clinical examination, chest and spinal radiography, spirometry, and diffusing capacity measurements. Twenty-seven of these patients also had body pletysmography, xenon 133 radiospirometry, and a test for bronchial hyperreactivity. Chest asymmetry was present in 29 patients (48%). Eleven patients (18%) had pectus excavatum, and one had a pectus carinatum deformity. Anterior asymmetry, pectus deformities, and a flat chest were more common among the patients who initially had a large diaphragmatic defect. Two patients underwent anterior thoracoplasty because of their deformities. Sixteen patients (27%) had significant scoliosis (Cobb angle > or = 10 degrees). Scoliosis was more common in patients with ventilatory impairment than in those with normal lung function. One patient required spinal stabilization, one with severe kyphoscoliosis remains under observation, and a patient with moderate scoliosis was treated with a spinal brace. The results of this study indicate that chest wall deformities and scoliosis are common among adults with repaired congenital diaphragmatic defects. In most patients the deformity is mild, but some will require surgery. Surveillance until adulthood is appropriate.

Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects.

Recent reports indicate a significant incidence of gastroesophageal reflux (GER) and other nonpulmonary problems after the repair of congenital diaphragmatic defects. Reports of follow-up through adulthood are few and based on a small number of patients. From 1948 to 1982, 107 of 164 patients (65%) treated at the authors' institution survived after repair of congenital diaphragmatic hernia or eventration. Sixty of the 107 survivors (56%) (mean age, 29.6 years; SD, 9.0 years) were interviewed and examined clinically. Forty-one of the sixty (68%) underwent upper gastrointestinal endoscopy. Early postoperative GER was recorded for 11 of the 60 patients (18%). Two of them underwent fundoplication because of an esophageal stricture. At the time of the follow-up study, 38 of the 60 (63%) reported symptoms suggestive of GER. Endoscopic or histological GER (esophagitis, Barrett's esophagus) was present in 22 of 41 patients (54%). No significant correlation between the initial severity of the diaphragmatic defect or neonatal postoperative problems and the late GER could be verified. Intestinal obstruction requiring hospital admission occurred in 12 of the 60 patients (20%), eight of whom had surgical treatment between 1 month and 20 years after repair of the diaphragmatic defect. GER and intestinal obstruction are common among patients who have undergone repair of a congenital diaphragmatic defect. Investigations for GER should be performed routinely during the follow-up of these patients.

Congenital eventration of the diaphragm - 45 years' perspective.

Fifty-five children with congenital eventration of the diaphragm were operated upon in a single tertiary-care children's hospital during the 45-year period from 1948 to 1992. The study was divided into three periods: 1948-1962, 1963-1977, 1978-1992. There were 14 patients in the first group, 26 in the second, and 15 in the third. Children later in the series presented earlier and with more acute symptoms. Mortality was related to severe pulmonary hypoplasia and associated anomalies, especially chromosomal defects: 14% during the first period, 27% during the second, and 7% during the third.

Barrett's oesophagus and perforation of gastric tube ulceration into the pericardium: a late complication after reconstruction of oesophageal atresia.

15 years after replacement of atretic segment with a gastric tube, perforation of an intrathoracic gastric tube ulcer into the pericardium, oesophago-pericardial fistula and severe mediastinitis developed in a 17-year-old male after reconstruction of oesophageal atresia using a retrosternal Heimlich-tube with a cervical oesophagogastric anastomosis. For six years he suffered from reflux oesophagitis and had Barrett's metaplasia in the cervical oesophagus. The patient had previously had one unsuccessful attempt to correct the oesophageal atresia at the age of two years using transverse colon. The two stage treatment included subtotal oesophagectomy, pericardiotomy, cervical oesophagostomy, gastrostomy, mediastinal and pericardial irrigation with antibiotics. After healing of the mediastinitis, the continuity of the alimentary tract was restored by using an isoperistaltic subcutaneous ileocolic segment. After a follow-up of five years the patient is well, without any oesophageal symptoms.

Long-term endoscopic and flow cytometric follow-up of colon interposition.

During 1963 to 1978 colon interposition was performed on 20 children. Early and late mortality accounted for 3 patients and two transplants failed. Of the 15 long-term survivors, subjective results were obtained from 12 patients 12 to 26 years after the reconstruction. Three patients had an excellent subjective result and 9 had a good result. Nine patients underwent esophagogastroduodenoscopy 12 to 26 years after the reconstruction. The endoscopic findings included: marked tortuosity or dilatation (6), pooling of bile or yellow bezoar (3), macroscopic colitis (3), and polyps in the distal anastomosis (2). Only two patients had a straight colon tube without mucosal changes or marked retention. Specimens for histology were obtained in each endoscopy. Colitis was found in 3 patients, 1 patient had gastric metaplasia in the transplant, 1 patient had dysplasia of colonic mucosa and an inflammatory colonic polyp, and in 4 patients histology showed normal colonic mucosa. Flow cytometric samples were obtained from 8 patients. Two showed an aneuploid cell population; the other 6 had normal diploid findings. Because of frequent endoscopic and histological changes, regular endoscopic follow-up of these patients is warranted.

Dimensions of the heart and great vessels in normal children. A postmortem study of cardiac ventricles, valves and great vessels.

Knowledge of normal heart dimensions is often needed in the planning of cardiac surgery in children. Attempts to define 'normal' have so far given varied and somewhat controversial results. The purpose of this study was to determine normal values for all heart measurements of clinical importance in children. Special interest was focused on the relationship between the diameters of the right and the left pulmonary artery (RPA abd LPA) and the descending thoracic aorta (DTA), defined as RPA/DTA+LPA/DTA, which is widely used in evaluating the feasibility of certain pediatric cardiac operations. The normal curve of this ratio during growth has not previously been presented in the literature. The observed data provide reliable normal values for the diameters of the great vessels and valves and the ventricular volumes of the heart.

Surgery for ventricular septal defect.

Of 255 patients undergoing closure of ventricular septal defect (VSD), 48% were younger than 2 years, 59% had associated cardiac and 26% non-cardiac abnormality, 13% had multiple, and only 29% isolated VSD. VSD was closed via the left ventricular apex in seven cases, without increased morbidity or mortality. The three early and six late deaths occurred in patients with complicated defects. Pulmonary vascular occlusive disease caused four deaths (1 early, 3 late). At follow-up (mean c. 3, range 1-11 years), 79% of the patients were well, 10% had cardiac symptoms and 7.5% had symptoms from associated noncardiac anomalies. Reoperation for significant residual VSD was required in 12 cases (4 single and 8 multiple VSD). Complete, pacemaker-requiring A-V block was found in four patients (none with simple VSD closure). It is concluded that 1) concomitant cardiac and non-cardiac lesions are common in VSD, 2) mortality is closely related to such lesions and to pulmonary vascular occlusive disease, 3) the latter is a rare, but real cause of death, 4) left ventricular approach need not increase mortality or morbidity, 5) significant residual VSD is rare after single, but common in multiple VSD, and 6) risk of complete A-V block after simple VSD closure is very low.

The rectourogenital connection in anorectal malformations is an ectopic anal canal.

Histological investigation of the rectal blind pouch and rectourogenital or rectoperineal connection was performed in 10 patients with high or intermediate anorectal malformations. Nine of the patients underwent postoperative manometric evaluation. In nine of the 10 patients, transitional epithelium typical of the normal anal canal could be found in the distal rectum or rectal end of the fistulous connection. The zone of transitional epithelium was aganglionic and showed abnormally strong acetylcholinesterase reaction. A positive rectoanal inhibitory reflex was found manometrically in all cases in which the distal rectal pouch was utilized in the reconstruction of the anal canal. The slow pressure wave activity of the reconstructed anal canal was characteristic of a normal anal canal. The manometric evidence strongly suggests that there is a functional internal sphincter in high and intermediate anorectal malformations. The present study shows that in anorectal malformations the distal rectal pouch with the fistulous connection is actually an ectopic anal canal.

Cervical Barrett's esophagus: a common complication of gastric tube reconstruction.

Upper gastrointestinal endoscopy was performed on 14 of our 18 long-term (more than 2 years) gastric tube esophagus survivors, with special attention paid to cervical gastric metaplasia. Barrett's esophagus was found in 10 patients. In eight cases, this could also be histologically verified. Three patients had esophagitis, but no verified gastric metaplasia. Isotope reflux studies were performed on six patients, all having both endoscopically and histologically shown pathology in the cervical esophagus. Reflux was provoked by putting patients in different positions. Only one patient showed gastrotubal reflux, and only in the prone Trendelenburg position. We conclude that Barrett's esophagus is a common complication of gastric tube patients, and is probably due to acid secreted by the tube itself. Life-long endoscopic follow-up of these patients is warranted.

Comparison of hollow fibre membrane oxygenators during cardiopulmonary bypass in children: Dideco Masterflo versus Terumo Capiox II.

This prospective study was planned to compare two different (reversed blood and gaseous compartments) hollow fibre membrane oxygenators, Dideco Masterflo and Terumo Capiox II, in order to find out which of these oxygenators was less injurious during cardiopulmonary bypass (CPB) surgery in small children. Twenty children underwent CPB surgery for complex congenital cardiac malformations. Ten patients were selected for each study group by matching age and weight. Oxygenators were the only variables in the CPB circuit, and special attention was focused on the kinetics of white blood cell count (WBC), complement C3, anaphylatoxin C3a, total haemolytic complement (CH100), C-reactive protein (CRP) and haptoglobin. There were no significant differences between the parameters studied at any sample time. However, there was less intense C3a (peak C3a levels 2506 +/- 1187 SD versus 4302 +/- 3958 SD; p = 0.19) generation, and also less intense CH100 consumption (percentual drop of initial CH100 values was 30% versus 55.2%) in the Dideco Masterflo group of patients. Moreover, postoperative respiratory treatment was somewhat shorter when using Masterflo oxygenators (2.4 +/- 2.41 SD versus 4.0 +/- 2.49 SD days; p = 0.16), although statistical significance was not obtained. These findings may be important in high-risk paediatric patients undergoing open-heart surgery.

Failure of the Nissen fundoplication to control gastroesophageal reflux in esophageal atresia patients.

During the 5-year period from 1981 to 1985, Nissen fundoplication was performed on 13 esophageal atresia patients. One patient with Down's syndrome died because of cardiac malformation 2 weeks after the operation and is excluded from the analysis. Nine of the remaining 12 patients had the usual malformation with distal fistula, while three had isolated atresia. In eight patients there was a long gap between the segments, and in five Livaditis myotomy was necessary. The median age of the patients at the fundoplication was 1.3 years (range, 4.5 months to 10.6 years). The main clinical manifestations were anastomotic stricture (six patients), respiratory complications (three patients), vomiting and difficulties in feeding (two patients), and esophagitis only (one patient). Altogether nine patients had preoperative distal esophagitis. Mean follow-up time was 4.1 years (range, 2.0 to 6.4 years). All patients primarily benefited from the operation. Routine control endoscopy 3 to 8 months after the operation showed a competent fundoplication in all patients. However, in five patients the reflux later recurred, and endoscopy revealed a partially disrupted fundal wrap and esophagitis. Four patients underwent refundoplication and one is waiting for it as of this writing. Four patients had Barrett's esophagus at the last endoscopic control. There was one late death due to cardiac failure. In conclusion, although the short-term results of Nissen fundoplication in esophageal atresia patients are good, the risk for late recurrence is high. Regular long-term follow-up is therefore necessary.

Iatrogenic, unexpected and other vascular rings in children.

Between May 1955 and July 1987, 33 children with a vascular ring compression syndrome were treated at this institution. There were 4 iatrogenic, 2 unexpectedly found and 27 symptomatic congenital vascular rings. Accurate diagnosis is based on a water soluble iodine contrast dye oesophagogram, bronchoscopy and angiography which are complementary examinations. All symptomatic congenital vascular rings were treated successfully without mortality, but the mortality was 50% if the lesion was iatrogenic in origin or unexpectedly found during palliative or corrective cardiac surgery.

Neuronal intestinal dysplasia.

A series of 21 patients with NID is presented. A histologic and histochemical picture of NID was seen in an heterogenous group of patients. NID was associated with bowel obstruction and/or perforation in six neonates and infants. One neonate died. During follow-up the bowel histology gradually normalized in four of the five patients. NID was found incidentally in four patients with anorectal malformations and two with Hirschsprung's disease. Three patients with Hirschsprung's disease and associated NID had chronic proctitis; one patient with an anorectal anomaly had chronic obstipation and megacolon and one proctitis. Two children with multiple endocrine neoplasia 2b syndrome and chronic obstipation had typical NID in their rectum biopsies, as did a 50-year-old woman with CIIP. The clinical heterogeneity of patients with NID suggests that NID may not be a distinct clinical entity but rather a reaction of the neuronal network of the bowel wall and could be caused either by congenital or secondary factors.

Duodenal atresia: late follow-up.

In this study, 41 randomly chosen patients aged 15 to 35 years (mean 22 years) were carefully examined. As primary operations there were 13 membrane excisions, five duodenoduodenostomies, 22 duodenojejunostomies, and one gastrojejunostomy. Twenty-eight patients were symptom-free, ten admitted some discomfort, three had major pains, including one with a history of duodenal ulcer. Reoperation for adhesion ileus had been performed in six patients, in the early postoperative phase in one instance. At late follow-up barium meals (N = 41) showed completely normal findings in two cases only, hiatal hernia in two, gastritis in three, duodenogastric reflux in 12, slight dilation of the duodenum with good emptying and no reflux in 16, a huge duodenal sac in nine, diminished peristalsis in eight, delayed emptying in five, slight luminal narrowing in three, duodenal diverticuli in nine, bezoars in two, and a polyp in the duodenum of one patient. Ultrasound (N = 35) revealed a gallbladder septum in one patient and a dilated common bile duct in another; in one subject the gallbladder was not visualized satisfactorily. Isotope biligraphy (N = 15) showed biliary reflux to the stomach in 12 cases. Endoscopy (N = 20) findings were: esophagitis (1), hiatal hernia (2), gastric mucosa in the lower esophagus (2), biliary reflux (9), gastritis (7), gastric polyps (2), dilated duodenum of variable degree (19), diminished peristalsis (4), marked retention (2), abnormal papilla (3), diverticuli (4), and a persistent membrane (1). Histology showed superficial gastritis in three patients. E coli was cultured from the duodenal juice in five patients and Candida found in two.(ABSTRACT TRUNCATED AT 250 WORDS)

Pancreatitis after open heart surgery in children.

Between January 1981 and March 1986, we found 54 children with abnormal serum amylase values or clinical pancreatitis after open heart surgery. Of these 33, had increased serum amylase values only, and 19 had increased serum amylase values in conjunction with clinical pancreatitis. Two patients had haemorrhagic pancreatitis identified at autopsy. The mortality was 42.9% (9/21) in patients with pancreatitis compared to 9.1% (3/33) with amylasaemia only. Pancreatitis is a serious complication after complex open heart surgery in children. The aetiology is probably of vascular origin, and routine serum amylase screening is recommended.