Ilmo Louhimo

Esophageal atresia: Primary results of 500 consecutively treated patients

During 1947-1978, 500 patients with esophageal atresia and/or tracheoesophageal fistula were treated at the Childrens Hospital, University of Helsinki. The proportion of different types of anomaly followed the usual distribution, the commonest being the type with distal fistula (88.2%). For analysis, the patients were divided into five phases, each consisting of 100 consecutively treated patients. The hospital mortality decreased from 81% in the first phase to 15% in the last phase. No patients were excluded, not even those cases with lethal associated anomalies. Many more severe cases were treated in the last two phases than in the first three. Patients with distal fistula were examined separately as were the patients without a fistula and those with only a tracheoesophageal fistula. With time, early diagnosis and early referral for treatment became a rule. This led to the policy that nearly all patients in the last phase had an early operation without staging. Gastrostomy was not considered necessary when early anastomosis was possible. In the last phase, only the transpleural approach was used and single-layer end-to-end anastomosis was favored. As a whole, the type of approach or anastomosis did not have significant effect on the results, except that the Sulamaa-type end-to-side anastomosis had the highest frequency for refistula. With time there was only a slight decrease in the incidence of anastomotic leak, but significant improvement in its management. Refistula as an early complication was seen only once in the last phase. As all anastomoses were routinely dilated, severe strictures were uncommon; there were only six in the series. The factor that probably improved the prognosis the most was better pulmonary care. The improved prognosis was also due to earlier referral, modern anesthesia, and intensive care. Postoperative pulmonary complications dropped from 92% in the first phase to 40% in the last. They were the most common single cause of death in the early series but caused no deaths among the last 100 patients with no associated anomalies. Low birth weight was an important prognostic factor early in the series but in the last phase the survival rate of under-2500-gm infants with no associated anomalies was 88%. The presence of severe associated anomalies remains the most important single cause of death of an esophageal atresia patient today. Some patients (eg, trisomy 18) are beyond the possibilities of surgical treatment. To improve the prognosis of the others, efforts in the treatment of their associated (especially cardiovascular) anomalies must be made. In the treatment of esophageal atresia itself, improvement may still be achieved in the number of patients using their own esophagus without replacement procedures. Finally, a new prognostic classification of esophageal atresia patients is suggested, excluding pneumonia as an index factor in the Waterston classification.

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.

Colon interposition or gastric tube? Follow-up study ofcolon-esophagus and gastric tube-esophagus patients

Between 1963 and 1980, 34 elective esophageal reconstructions were performed on 29 esophageal atresia patients. Five patients needed two reconstructions. Among 20 colon replacement procedures there were 2 early deaths and three transplants failed. In 14 gastric tube reconstructions there was no mortality, but two tubes failed. The mean follow-up age of the 15 colon esophagus patients was 11.8 yrs, while for the 12 gastric tube patients it was 5.6 yrs. One death occurred during the follow-up period in each group. Late complications occurred in 7/15 of the colon and 3/12 of the gastric tube groups. Most of the serious complications occurred within 3 yr after surgery. The previously reported malabsorption following colon interposition seemed to be transient. At follow-up all but 3 patients with no significant other anomalies were within two standard deviations of the mean of height and weight. All were satisfied with their new esophagus. Nine out of 14 of the colon and 7/11 of the gastric tube groups were without symptoms, the others having only minor complaints. It is concluded that both colon replacement and gastric tube are satisfactory methods for esophageal reconstruction, and the long-term function seems equally good. However, the gastric tube procedure is easier to perform, has less mortality and fewer complications than colon replacement.

Anorectal malformations: results of treatment and long-term follow-up in 208 patients

During a 20-year period (1964–1983) 208 patients with anorectal malformations (143 males, 65 females) were treated; 98 had low and 110 had high or intermediate malformations. Associated anomalies were detected in 67.8%. The overall mortality in the series was 14.4%; most deaths were related to associated anomalies and/or prematurity. Four different procedures were used for high and intermediate anomalies: (1) abdominoperineal (18 cases); (2) sacroabdominoperineal (38); (3) sacroperineal (8); and (4) posterior sagittal anorectoplasty (7). Neonatal anoplasty was used to correct male low anomalies. Female low and intermediate anomalies with vestibular fistula were treated with anal transposition. More than one-half of the patients with high or intermediate anomalies had complications related to surgical therapy. Major secondary surgery was performed in 41 cases (12 resections of megarectum, 29 late sphincter reconstructions). Of the living patients, 90% (159/178) were followed up for 5–25 years. A majority (93%) of the patients with low anomalies had a good outcome at the last follow-up examination. In patients with high and intermediate anomalies continence improved with time; the greatest improvement occurred after 10 years of age. At the last follow-up examination 73% of the patients with high or intermediate anomalies had acceptable social continence. Manometric evaluation revealed a rectoanal inhibitory reflex in all assessed patients with a low anomaly, indicating a functioning internal sphincter. In patients with intermediate and high anomalies a rectoanal inhibitory reflex was found only when full-thickness terminal bowel had been used in the reconstruction (by perineal and posterior sagittal repair); presence of the reflex correlated with superior fecal control. Acceptable fecal continence may be achieved in the majority of patients with anorectal anomalies. Operative methods that optimally utilize the voluntary sphincters and preserve the potential internal sphincter in the terminal rectal pouch seem to facilitate earlier development of fecal continence.

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 Downs 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 Barretts 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.

Late gastrointestinal complications in patients with colonic replacement of the esophagus

Abstract Follow-up study of six patients with esophageal atresia treated by colonic replacement revealed growth failure in five cases. This retardation of growth was not evident during the gastrostomy feeding period, but only after colonic transplantation. Absorption studies showed severe fat malabsorption in three patients. The patient with normal growth curve had another severe late gastrointestinal complication: complete gastric obstruction attributable to food retention. Gastric retention in lesser degree was found in three other patients. The significance of these findings is discussed and the importance of careful follow-up of patients with colonic replacement of esophagus is emphasized.

Livaditis Myotomy in Long-Gap Esophageal Atresia

During 1973 to 1983, Livaditis myotomy was performed on 12 esophageal atresia patients in whom anastomosis otherwise would have been impossible. Ten patients had the usual malformation with a distal fistula, while two had an isolated atresia. Four patients belonged to Waterstons risk group A, 4 to group B, and 4 to group C. In 11 cases anastomosis was permitted by myotomy. In one patient anastomosis was impossible even after myotomy. This baby weighed 1,380 g and she also had trisomy 18 with severe cardiac and renal anomalies leading to death. One patient had a refistula and died of pneumonia, she also had a severe congenital cardiac malformation. There were no anastomotic leakages to free pleural cavity nor complications related to the myotomy. The ten survivors were followed up for a mean period of 5.4 years (range 1.6 to 11.3 years). The subjective results at last follow-up according to the criteria of Desjardins were excellent in eight and good in two patients. Esophagograms showed no strictures, but two patients had a myotomy pouch.

Bowel function and quality of life in adult patients with operated Hirschsprung's disease

Fecal continence and quality of life were evaluated by a questionnaire in 100 patients (mean age 31 years, 88 males, 12 females) who had undergone surgery for Hirschsprungs disease (HD) during the years 1950–1975. The operative methods were Duhamel retrorectal pull-through or modified Duhamel operation in 71 patients, Swenson rectosigmoidectomy in 20, State-Rehbein anterior resection in 5, and Soave endorectal pull-through in 4. The postoperative follow-up period ranged from 15 to 39 years. Fecal continence was assessed by a score described by Holschneider. Eighty-one healthy people with a similar age and sex distribution as the patients were used as controls. All controls and 91 patients had good fecal continence scores. Of the 9 patients who had fair scores, 3 were mentally retarded and 3 had postoperative anastomotic complications. If these 6 patients with an obvious cause for deficient anal function are excluded, there was no statistically significant difference in fecal continence between the patients and the controls. Equally good continence outcomes were achieved with all the operative methods. In adulthood, only 1 patient had chronic constipation. However, during childhood constipation was the main problem in 40 patients who developed a recurrent rectocolonic septum following the Duhamel operation. Constipation subsided after recrushing of the septum. None of the patients had urinary incontinence. All the patients with a good continence outcome reported no limitations in their occupation, social life, or physical activities. We conclude that in adulthood the fecal continence and quality of life of most patients with operated HD do not differ from those of healthy adults.

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.