Results of the operative treatment of gastroesophageal reflux in childhood with particular focus on patients with esophageal atresia.

AIM: Although the literature on fundoplications in childhood is relatively extensive, only few reports exist which correlate the rate of complications with the primary disease. Other important questions such as the incidence of postoperative dumping syndrome or Barrett's esophagus in childhood are barely treated. Nor have operative techniques such as single or double-row cuff sutures or the benefit of performing pylorotomy or pyloroplasty in addition to fundoplication been investigated with respect to their recurrence rates. In particular studies on laparoscopic fundoplications tend to be generally confined to a discussion of the feasibility of the endoscopic procedure, the duration of the intervention, the length of the hospital stay and the costs. The study presented here aims to analyze such still unanswered questions listed above for open fundoplication procedures, including an analysis of the authors' own patient population, and to discuss the questions together with the most important reports in the literature. The aim is to create a basis for later studies which will compare conventional and laparoscopic fundoplications. METHODS: In the period between 1993 and 2005, 160 children underwent a fundoplication procedure. From 2003 onwards, fundoplications were carried out laparoscopically. The data of 148 patients were analyzed, some of them on the basis of clinical follow-up and some on the basis of an extensive questionnaire and among others with the help of the parents' support group KEKS. RESULTS: The underlying disease in 87 patients was reflux disease stemming from esophageal atresia, previous diaphragmatic operation in 8 children and mental retardation with swallowing difficulties in 30 patients. Only 23 patients suffered from isolated gastroesophageal reflux disease without an underlying primary disease. Intraoperative complications occurred in 4.6 % of patients with esophageal atresia (EA), while the rate for the remaining collective of patients was 1.6 %. Postoperative complications were observed in 10.3 % of the children with esophageal atresia and in 8.2 % of the other cases. The recurrence rate was 16.1 % in the children with EA and 6.5 % in the other cases. Dysphagia and/or stenosis occurred in 17.2 % and 6.5 % of children, respectively, and dumping syndrome was observed in 18.3 % of the EA group and only in 1.6 % of the comparison group. An evaluation of 79 esophageal biopsies showed no difference between patients with EA and the comparison group concerning the degree of histological changes. When evaluating the suturing technique, it was found that a double-row fundus suture was more effective in preventing reflux recurrence than a single-row suture but also resulted in an increased rate of dysphagia. When considering the not infrequent occurrence of dumping syndrome, it was found that drainage operations such as pyloroplasty or pyloromyotomy are only indicated for reduced gastric motility. The data of the children without EA, who initially served as a comparison group, was analyzed further. Four summaries of the current literature in table form complete this discussion. CONCLUSION: Particularly in children with EA, Nissen fundoplication cannot be considered a procedure with few complications. The problems resulting from disturbed gastric and esophageal motility should not be underestimated nor should the postoperative occurrence of dumping syndrome. Postoperative long-term follow-up until the patients reach adulthood is absolutely necessary to ensure that development of a Barrett's esophagus is not overlooked.

Familial form of hirschsprung disease: nucleotide sequence studies reveal point mutations in the RET proto-oncogene in two of six families but not in other candidate genes.

Hirschsprung disease (HSCR; McKusick 142623) or aganglionic megacolon is a frequent (1 in 5,000 live births) heritable disorder of the enteric nervous system. By haplotyping with a variety of microsatellite markers, by amplifying all 20 exons of the RET proto-oncogene and by applying a direct DNA sequencing protocol, we have analyzed the DNA from HSCR patients in 6 different families. In one family with a joint occurrence of HSCR and FMTC (follicular medullary thyroid carcinoma), we have identified a mutation in codon 609 in one out of 6 cysteine residues encoded in exon 10 of the RET gene. This C609R point mutation has not previously been reported to cause HSCR. In 2 of the HSCR patients described here from different families, we have found a mutation in exon 2 (R77C) and a silent mutation in exon 3 (Y204Y), respectively, in the extracellular part of the RET proto-oncogene. In introns 2 and 17 of the RET proto-oncogene in 2 families, we have detected single nucleotide exchanges that are probably polymorphisms with unknown, if any, relations to HSCR. The DNA sequences of 5 further genes (GDNF, GDNFRalpha, EDN3, EDNRB, and NTN), that may contribute to the development of HSCR, have not shown mutations in the patients analyzed so far. In 2 of the reported families with several affected children and one grandchild, sequence analyses revealed no mutations in the coding regions of any of the candidate genes analyzed.

Reverse smooth muscle plasty: a new method of treating anorectal incontinence in infants with high anal and rectal atresia.

A new technique of treating anorectal incontinence in infants with high anal and rectal atresia is described. In the first stage of this new operation an abdominosacroperineal pull-through procedure according to Rehbein, Romualdi, and Kiesewetter is performed. In the next step of this new method the circular and longitudinal muscle layers of the pulled-through colon are turned back 180 degree and fixed to the pulled-through bowel. The mucosa of the turned back bowel is removed. Then the whole muscle cuff is re-pulled into the puborectalis sling. Pressure studies as well as X-ray investigations showed a good sphincter function of the newly established internal sphincter equivalent.

Innervation patterns of the rectal pouch and fistula in anorectal malformations: a preliminary report.

The innervation patterns of the rectal pouch and fistula of 52 children with anorectal malformations were investigated. Posterior sagittal anorectoplasty was used for intermediate and high anomalies; for the latter it was combined with an abdominal approach. Perineoproctoplasty was performed for low anomalies. The specimens were investigated by acetylcholinesterase staining, lactate dehydrogenase, and succinyldehydrogenase reaction. They consisted of fistula material only in 23 patients and of parts of the rectal pouch in 29. Fourty-four patients (84.6%) had follow-up, and information of bowel movements and continence was obtained after a mean of 3.3 years. Abnormal innervation patterns were found in 96% of the specimens. All fistulas were found to be aganglionic, including the adjacent part of the rectum involving the internal sphincter equivalent. Classical aganglionosis was found in 31% of the rectal pouch specimens, hypoganglionosis in 38%, neuronal intestinal dysplasia (NID) type B in 14%, and dysganglionosis in 10%. All patients with severe constipation or soiling at the time of follow-up had some histopathological correlation. Of the 25 patients for whom the specimens had consisted of rectal pouch material, nine (31%) had severe constipation. All four patients with a low-type malformation who had follow-up and pathological innervation patterns in the rectal pouch suffered from severe constipation; this was true of only five of the 19 children with intermediate or high malformations (P < .05). However, numerous pathological innervation patterns had been identified in patients who had normal bowel function at the time of follow-up. It is concluded that partial denervation of the rectum may not be the only cause in the pathogenesis of constipation after posterior sagittal anorectoplasty and perineoproctoplasty. The high frequency of neuronal intestinal malformations in the rectal pouch may be related to the higher frequency of bowel disturbances in patients with low malformations, in whom the resection was less radical. However, the clinical course is not necessarily related to specific histopathological findings. In the authors' opinion, the recommendation to use the distal rectal pouch and parts of the fistula in the reconstruction of anorectal malformations should be reconsidered.

The development of anorectal continence and its significance in the diagnosiso of Hirschsprung's disease.

The development of the anorectal continence reaction was studied in a series of 138 electromanometric recordings performed in 62 newborn and six premature infants. Forty-five babies were observed in a longitudinal study. In 11% an internal sphincter relaxation was observed at the time of birth. The same reflex was present in about 50% after the third day and in all infants after the 12th day of life. The development of a rectal continence reaction depends on an intact internal sphincter relaxation. The puborectalis muscle, is fully functional at birth. On the basis of these findings electromanometric recordings can be used to exclude aganglionic megacolon as early as the first day of life. An absent internal sphincter relaxation reflex, is diagnostic only after the 12th day of life.

Quality of life more than 20 years after repair of esophageal atresia.

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.

Necrotizing enterocolitis (NEC)--mortality and long-term results.

From 1974-88 73 patients were treated for necrotizing enterocolitis (NEC) in the surgical department at the Children's Hospital of Cologne. 70% of these had other complaints and 77% were premature babies, of which 30% were small-for-dates. A greater than expected proportion of deaths occurred in babies not of low birth-weight: Of those above 2500 g 35% died and of those under 1500 g 20%. Initial signs and symptoms became apparent in 80% within the first two weeks. The most frequent signs were distended abdomen (81%), diffuse tenderness (46%), bloody stools (39%) and absent peristalsis (39%). Radiology showed edema of the bowel wall in 49%, free air in the abdomen in 38% and intestinal pneumatosis in 34%. It remains questionable whether peritoneal lavage could lead to earlier diagnosis and referral of the sick children to the hospital. The course of the disease was less severe when babies had received breast milk. Colon and distal ileum were involved most frequently. Laparotomy showed perforations of the bowel in 39 patients (64%), inflammatory alterations in 18 and necroses in 12. The survival rates improved within the period of 1974-88 from 64 to 79%. Thirty-two of the 51 survivors were available for follow-up: 9 patients (28%) had more than 4 stools/day, 8 (25%) were underweight, 8 (25%) suffered from food intolerance, 5 (16%) from diarrhoea, 4 (13%) from limited exercise tolerance. Physical retardation could only be seen in 3 children under the age of 2.

Training in pediatric surgery--a comparison of 24 countries in Europe and other countries around the world.

What is the training in pediatric surgery like, how is pediatric surgery defined in other countries and what are the possibilities of quality control for training in pediatric surgery? The results of an inquiry together with information about training in pediatric surgery in 24 countries are summarised to show the different possibilities of organising training in pediatric surgery inside and outside Europe. The number of trainees in pediatric surgery (ranging from 0 to 339 trainees per country), the density of pediatric surgeons (ranging from 424 to 35 714 live births/year per pediatric surgeon) and the number of Centres of Pediatric Surgery (ranging from 4167 to 65,000 live births or 450,000 to 5,300,000 inhabitants per Centre) varies a lot in the different countries. Countries with a higher density of pediatric surgeons often also have a higher number of trainees irrespective of the birth rate and the number of inhabitants within the country, indicating a good infrastructure for pediatric surgery. In 87.5 % of the countries pediatric surgery is recognised as a specialty. The mean duration of training is about 6 years and 3 months, excluding the countries where it is necessary to become a fully accredited general surgeon before doing pediatric surgery. The mean duration of stay in general surgery is 2 years and 10 months. In one third of the countries it is obligatory to spend some time (3 to 12 months) in pediatrics. An elective or a compulsory period of time (1 - 6 months) in other specialties such as orthopedic surgery, plastic and reconstructive surgery, thoracic surgery and urology exists in 41.7 % of the nations. The most common subspecialties within pediatric surgery practised during the training by the resident are abdominal surgery, thoracic surgery, oncological surgery, head and neck surgery and urology. Possible means of control to guarantee a high quality of training include a defined time of stay in each subspecialty, the number of operations done by the trainee or the number of cases treated by the resident in the respective subspecialty and the distinction of different levels of surgery done by the trainee during his residence. In many cases the type of control is not specified.

Ventriculoscopic implantation of ventricular shunts in children with hydrocephalus.

A modified technique of ventriculoscopy-aided implantation of ventricular shunts in children with hydrocephalus or intracranial cysts is presented. A 1 mm 0 degrees optic is introduced into the ventricle catheter and the shunt is positioned under video control. Finally, the optic is removed and the catheter left in place. Four endoscopy-aided implantations were performed with excellent visualization and without technical failure, the smallest patient weighted 680 grams.

Proximal segment histology of resected bowel in Hirschsprung's disease predicts postoperative bowel function.

The authors present an overview of 101 patients operated for Hirschsprung's disease by Rehbein procedure. Special attention is directed to the histomorphological findings in the proximal segment of the resected bowel specimens. A strong link was confirmed between these specific features and postoperative bowel function. A histologically regular proximal bowel segment generally predicted good postoperative bowel function with a constipation rate of 10.5%. In patients with intestinal neuronal dysplasia (IND) of the proximal segment the overall clinical result remained unchanged, although the constipation rate rose to 17.8%. Postoperative bowel function was seriously affected in cases characterized by proximal segment hypoganglionosis with a constipation rate of 23.1% and an additional 7.7% with encopresis. Finally, the distinct group of children with aganglionosis of the proximal segment followed a complicated postoperative course with secondary bowel resections and recurrent episodes of enterocolitis. In addition, the authors state their general observation that histological findings become less important whenever a more extensive resection than left hemicolectomy is required. Discussing the results, guidelines are given to further patient treatment once the particular proximal segment histology is diagnosed.

Long-term follow-up of children with diaphragmatic hernia.

From 1962 to 1988, 147 neonates were admitted for operative repair of CDH to the Division of Pediatric Surgery in the Children's Hospital of Cologne. Follow-up studies were performed on 45 patients ages 1 to 25 years representing 54.2% of the 83 survivors. 18 patients (40%) were entirely without any complaints, 11 patients (24.4%) had increased rates of respiratory infections. Especially in early childhood they suffered from obstructive alterations in the respiratory tract. No restrictive pulmonary changes were observed. Scintigraphic ventilation tests were performed on 44 cases. 34 (77%) tests indicated absolutely normal results. Some rare local deficits of lung ventilation were based on adhesive anatomic alterations of the thoracic skeleton and the diaphragm. The lung function tests conducted in 29 persons ages 6 to 25 years revealed that the vital capacity and the forced expiratory volume were all normal. We found an increase of the intrathoracic gas volume in 11 infants (disposition to pulmonary inflation). In 9 cases we observed a decrease in the mid expiratory flow curves and in 6 patients we measured an increase in the specific resistance of airways (tendency towards obstructive airway alterations). Nevertheless even former hypoplastic lungs revealed a good extensibility.

The relative proportions of type I and type II muscle fibers in the external sphincter ani muscle at different ages and stages of development--observations on the development of continence.

The external sphincter ani muscles were partially or completely removed from 11 fetuses, 3 infants, 2 children and 12 adults, none of whom had had anorectal disease and all of whom had died of diseases not affecting the anorectal organ of continence. Muscle fiber-typing was carried out. In the fetuses ages 26-40 weeks, we found a predominance of Type II (rapid) fibers. With increasing gestational age Type II fibers started to diminish along with a simultaneous increase in Type I (slow) fibers. At the same time, stromal restructuring took place. Three infants, ages 1, 2 and 10 months, showed further increases of Type I fibers, until finally, in the two older children studied (5 and 8 years), there was a predominance of Type I fibers. This persisted in adults between ages 56 to 70, but after age 70 Type I fibers diminished, so that the ratio of slow to rapid fibers in the 78 to 81 year-old group was 3:2. This study shows that in contrast to other skeletal muscles the fiber distribution in the external sphincter ani muscle is not established from the beginning, but is the result of a developmental process. The predominance of Type II (twitching) fibers explains the state of reflex continence of the young infant. With increasing maturation of tonic Type I fibers, an additional voluntary component to continence is made possible with the help of the supporting pelvic musculature. This maturation is determined by the increasing strain on the pelvic floor as the child learns to sit and walk.(ABSTRACT TRUNCATED AT 250 WORDS)

Results of total and subtotal colon resections in children.

AIM: The treatment of long-segment neuronal intestinal malformations confronts the paediatric surgeon with the problems of diagnosis, suitable surgical methods and postoperative care. The evidence based only on ganglion cells is inadequate to decide about the required extent of resection and does not exclude hypoganglionosis and disseminated dysganglionosis. For the surgical treatment, pouch procedures as well as the usual resection techniques according to Rehbein, Soave, and Duhamel are discussed. Since studies with greater numbers of patients are rare, we present here our own results. METHODS: 48 patients with long segment intestinal malformations were treated in our hospital between 1990 and 2000. A total of 35 patients were examined 1.5-6 years after definitive surgical therapy. Rehbein's anterior resection was performed in all cases. RESULTS: Our findings showed that the surgical treatment with Rehbein's technique offers good results, both with respect to complications as well as to the postoperative course, although a 4 cm long aganglionic segment remains in situ. We found that results were better after ascendorectostomy (n = 22) compared to ileorectostomy (n = 11). Earlier publications of this group (13) show that the histology of the proximal resection margin is decisive for the prognosis. Hypo- and aganglionic segments should be completely resected while short IND segments of the colon or terminal ileum may remain in situ. However, the additional effect of the aganglionic segment of the distal rectum and the decreased peristaltic flow of the pre-anastomotic bowel has to be taken into account. Further investigations are required to find out whether a combination of Soave's endorectal pull-through with a remaining neuronal dysplastic segment proximal to the resection margin may give better results or if the frequency of postoperative enterocolitis and incontinence increased in cases of long segment intestinal neuronal malformations. Accurate diagnosis of myenteric plexus is decisive for an optimal treatment and therefore, considering our results, it is essential that in case of newborns getting to hospital with colon obstruction and suspicion of neuronal intestinal malformation full thickness biopsies from the distal and proximal colon may be taken simultaneously with the enterostomy. Generally ileostomy is performed in patients suspected of long-segment neuronal intestinal malformations. Mucosa suction biopsies from the distal and proximal stoma side are less informative compared to full thickness biopsies.

Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch.

The results of surgery for anorectal malformation (ARM) in 536 children treated between 1962 and 1993 are presented. Before 1985, patients underwent an abdominoperineal pullthrough for high and intermediate type of ARM, whereas cut-back procedures or perineoproctoplasty were performed in low type. Since 1985, we have changed our policy and performed a modified posterior sagittal anorectoplasty (PSARP) for intermediate type of ARM, eventually combined with an abdominal mobilization for high type. Perineoproctoplasty was reserved for low type of ARM. Data of 143 patients of Group 1 and 85 patients of Group 2 were available for follow-up. Continence was compared using a clinical score. Good or fair continence was found in 82% of patients operated on before 1985 (low type 85% good, 15% fair, intermediate type 17% good, 50% fair, high type 12% good, 37% fair), comparing to 93% in children treated after 1985 according to the new regimen (low type 88% good, 6% fair, intermediate type 59% good, 35% fair, high type 43% good, 47% fair). Liquid stools and inability to retain bowel contents were the main problem in the older series, whereas incontinence could often be attributed to constipation with overflow incontinence in the recently treated group of patients. Analysis of the innervation of fistula and distal rectal pouch in a prospective study comprising 40 of our most recent patients showed normal innervation in only 5% of patients, whereas 66% had neuronal intestinal malformations (NIM) including aganglionosis, NID and hypoganglionosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Ultrastructural features of nerve fascicles and basal lamina abnormalities in Hirschsprung's disease.

Although the pathogenesis of Hirschsprung's disease (HD) is not completely resolved, both the absence of nerve cells and the hypertrophy of nerve fascicles within the aganglionic colonic segment have been attributed to an abnormal intestinal microenvironment. Studies on animal models for HD revealed an altered ultrastructure of ingrowing nerve fascicles and abnormalities of basal laminae (BL). Therefore, the purpose of this study was to examine the ultrastructure of hypertrophied nerve fascicles in human HD with special reference to structural abnormalities of BL. Colonic specimens were obtained from patients with HD (n = 10) and controls (n = 5) and processed for electron-microscopical examination. Hypertrophied nerve fascicles were characterized by a prominent perineural sheath surrounded by large amounts of collagen bundles, a collagen-filled endoneurium, vasa nervorum and abundant glial cells of extraenteric ultrastructure, which were arranged in mono- or oligoaxonal units and frequently displayed different stages of myelination. As these ultrastructural characteristics resembled typical features of extrinsic nerves and were similar to those observed in subserosal nerves, the prominent intramural nerve fascicles were considered to be of extraenteric origin. Most likely their overabundance contributes to the functional obstruction of the terminal colon. Morphological abnormalities of BL encountered in the aganglionic colonic segment consisted of an extensive multilamination of BL surrounding glial processes and an irregular thickening of BL surrounding perineurocytes and smooth muscle cells of the muscularis mucosae. Similar alterations in BL have also been described in inherited peripheral and diabetic autonomic neuropathies and attributed to reactivated schwann cells. Thus, the overproduction of BL material within the hypertrophied nerve fascicles in HD may reflect an increased activity of proliferating glial cells. Since the smooth muscle cells of the muscularis mucosae showed abnormalities of BL similar to those observed in murine models for HD, it is suggestive that also in human HD the aganglionic colon is affected by a disturbed intestinal micro-environment impairing the neuronal colonisation and promoting the ingrowth of extrinsic nerves. The ultrastructurally observed alterations in BL of both neuronal and non-neuronal cells, as well as the increased amount of perineural and endoneural collagen provide further evidence that extracellular matrix components are abnormally distributed and overproduced within the bowel wall of patients affected by HD.

Cost analysis of laparoscopic cholecystectomy in children.

A detailed analysis of the hospital-related costs of laparoscopic cholecystectomy in children was performed. Data on 10 laparoscopic cholecystectomies were collected prospectively. Pre-, intra- and postoperative variables were assessed by standardized questionnaires. The hotel costs and costs for nursing, physicians, medicaments and equipment were calculated in detail. Reusable instruments were assumed to be used for 50 operations per year over 5 years and the costs for disposable instruments were calculated for comparison. In addition, the data of 10 children who underwent open cholecystectomy were analyzed retrospectively. The mean hospital costs for one laparoscopic cholecystectomy was 3685 DM. The costs for the operation itself represented 36.5% of the hospital costs and were mainly due to expenses for surgeons and nurses. The costs for laparoscopic equipment and instruments represented only 8.5% of the total costs. If an open procedure instead of a laparoscopic operation had been performed with a similar duration of hospital stay, the hospital costs would have been reduced by 425 DM (11.5%). In laparoscopic cholecystectomy the costs for medical treatment in addition to the operation were 32.8% of the total costs and were mainly due to expenses for nursing and physicians, which added up to 165 DM per day. The hotel costs represented 30.7% of the total costs and added another 189 DM per day. Therefore, potential savings in the operation theatre are limited and most effective savings may be achieved by shortening the hospital stay. The use of disposable instruments would have increased the costs by 844 DM and already 20 operations per year would have been cheaper performed with reusable compared to disposable instruments. Reusable instruments are recommended. Additional 60 minutes operating time cost 312 DM (5.20 DM/minute) and therefore, laparoscopic training courses for surgeons may be cost-effective in order to reduce the costs for training in the operation theatre.

A complex, high cloacal malformation: case report.

Cloacal malformations are rare, complex inhibitional anomalies of early embryogenesis. We report a patient with a cloacal malformation in which a septate vagina and a rectal fistula emptied through a common orifice onto an exstrophic bladder plate. Additional anomalies included an omphalocele and malformations of the upper urinary tract and the lower extremities, skeleton, and vertebral column.

Paediatric surgical reality in Germany and visions for the future.

Over the past 2 centuries paediatric surgery has developed into a well-recognised, independent surgical speciality comparable to plastic surgery or thoracic and vascular surgery. About 550 members of the GAPS (German Association of Paediatric Surgery) annually treat about 2-3% of all 0-15-year-old children of the FRG and operate 32.5% of all children who need a surgical intervention in paediatric surgical centres. There are 83 paediatric surgical centres in the FRG. Most of them perform more than 2000 operations per year. Three newer developments are likely to influence the future of paediatric surgery in Germany: 1. demographic factors, particularly the massive reduction of the reproduction rate from factor 1 to 0.59; 2. the new German National Health Care System, called "Strukturreform 2000", which will introduce the Australian DRG system and a new administrative structure permitting only a restricted care of the patients under tight economic control; and 3. the European regulations of the UEMS/EBPS which the national Landesärztekammern will be required to adopt.

Surgical methods for anorectal malformations from Rehbein to Peña--critical assessment of score systems and proposal for a new classification.

In this review article the surgical techniques of Rehbein, Romualdi, Kiesewetter, and Peña/De Vries are compared and corresponding surgical steps are pointed out. Peña/De Vries have adopted many surgical steps from Stephens and further standardized this technique. The results of recent postoperative examinations are presented with regard to the surgical methods of Stephens, Rehbein, Romualdi, Kiesewetter, and Peña. In that connection, the paper makes it clear that comparison of postoperative studies is not possible because the authors evaluate too small numbers of cases, use different scoring systems, do not indicate individual steps of their surgical techniques and do not consider the height of the atresias. The reference to the type of fistula that can be found in recent literature unfortunately does not provide a common basis for comparative studies either. Therefore, the paper suggests complete abandonment of scoring systems and classification of postoperative continence results instead according to the therapy that has to be applied. In addition, it again stresses the importance and necessity of adhering to the Wingspread classification.

Biliary atresia--a 25-year survey.

In the past 25 years, from 1963 to 1988, 90 children from the Department of Pediatric Surgery of the Pediatric Hospital in Cologne, Germany were treated for biliary atresia. Of these, 47 had purely extrahepatic bile duct lesions, 21 had purely intrahepatic bile duct lesions, and 22 had both extrahepatic and intrahepatic bile duct lesions. Forty-five of the children underwent a drainage operation, whereas the remaining 45 children underwent no surgery at all or simply a diagnostic laparotomy. Until 1966 hepato-jejunostomy with implantation of artificial bile ducts was conducted in 12 cases. Later, cholecystoduodenostomy was performed 4 times and hepatoporto-jejunostomy according to Kasai-Kimura 29 times. The latter was performed either without and enterostomy (n = 16) or with an enterostomy in the respective intestinal loop (n = 13). 27 patients survived (30%). If only the children with intrahepatic bile duct hypoplasia are considered, the survival rate was 12 out of 15 patients (80%). Eight children (27.5%) of the 29 with hepatoporto-jejunostomy are still alive today. This survival rate, compared with the survival rate of the total, is comparably large with 27.7%. Current data from the 23 surviving patients was retrospectively gathered in our hospital or was collected from outside the establishment. It was analyzed with regard to prognosis and long-term results. Only in 5 of the 29 cases of children with hepatoporto-jejunostomy could a lasting postoperative biliary flow be achieved. Only one of the children can be classified as completely healthy in regard to his liver.(ABSTRACT TRUNCATED AT 250 WORDS)