Diagnosis and therapy of ultrashort Hirschsprung's disease.

BACKGROUND: Although ultrashort Hirschsprung's disease (UHD) was enzyme-histochemically characterised about 35 years ago, its existence is still often ignored. The aim of this study is to summarise the clinical diagnostic, incidence, gender ratio, morphological characteristics, and therapy over 15 years. METHODOLOGY: The reliable diagnosis of suspected UHD requires a minimal enema of contrast medium to exclude Hirschsprung's disease (HD). In UHD during pressing or crying no reflux of contrast medium is observed. Final proof of UHD is an enzyme-histochemical biopsy examination of distal rectal mucosa. The biopsies must demonstrate submucosa and be taken from the dentate line and 1 cm, 2 cm, 4 cm and 6 cm above the dentate line. The cryostat sections must be cut 15 microm thick; this thickness is reduced to 4.5 microm by the thawing, spreading and drying of the sections on microscope slides. A reliable diagnosis of UHD needs an enzyme-histochemical acetylcholinesterase reaction of native sections of rectal mucosa. RESULTS: UHD develops with first symptoms of chronic constipation in the second half of the first year of life. The chronic constipation proves to be therapy resistant. In HD constipation occurs in the first weeks of life or after weaning. In contrast to HD, no nerve fibres with increased AChE activity are observed in the lamina propria mucosa. Nets of nerve fibres with increased AChE activity can be found only in the muscularis mucosa and the musculus corrugator cutis ani (MCCA). The therapy of choice has proven to be a partial myectomy of the distal internal sphincter if dilatation of the internal sphincter was ineffective. UHD is either limited to the anal ring, or extends 3 - 4 cm into the distal rectum. Over the past 15 years, UHD had in our series an incidence of 13.4 % of all aganglionoses. The gender ratio of girls to boys was 1 : 2. CONCLUSION: UHD is reliably diagnosed by an AChE reaction in native biopsy sections from the anocutaneous transitional zone and, potentially, from 3 - 4 cm above the pectinate line. As UHD is always accompanied by aganglionosis of the distal internal sphincter, an increase in AChE activity is observed in the nerve fibres of the MCCA. The therapy of choice is a partial myectomy of the distal internal sphincter.

Long-term results of Rehbein's procedure: a retrospective study in German-speaking countries.

AIM: In contrast to other countries, no collective study of Rehbein's procedure in German-speaking nations has been performed. Therefore, our intention was, analogously to Goto and Ikeda's (10) Japanese study in 1984, Kleinhaus's (13) study on Swenson's procedure in 1979, Bourdelat's (2) French-Canadian investigation into Duhamel's technique in 1997 and Martuciello's (11) and Teitelbaum's (16) follow-up in the year 2000, to perform a follow-up study of Rehbein's technique of deep anterior resection. METHODS: The data of 200 patients from 22 German-speaking centers in Switzerland, Austria and Germany were collected. These data were gathered by questionnaire and the children were followed up in the individual participating hospitals for at least 3.5 years after the procedure. The procedure was performed between 1993 and 1997, over a 5-year period. The questionnaire contained 74 items including anamnestic data, diagnostic postoperative treatment and reoperations. RESULTS: Concerning the incidence of anastomotic leaks and resolving anastomotic strictures there was no significant difference between the results in our series and those of the collective analyses made by Hofmann von Kap-herr (7), Holschneider (9) and Sherman (18). In 6.6 % of the 191 patients an anastomotic leak and in 9.9 % a rectal stricture, which had to be dilated, was observed. Concerning late complications, 22.8 % of the children suffered from constipation, 4.3 % from encopresis, 10.6 % from enterocolitis and only 0.5 % from enuresis. The frequency of constipation diminishes over the years. A comparison of the different large series in the literature clearly shows that the incidence of constipation is higher after Rehbein's procedure and the frequency of urinary incontinence and encopresis higher following Swenson's, Soave's and Duhamel's techniques. The incidence of enterocolitis is less after Rehbein's procedure than after Swenson's, Soave's and Duhamel's techniques. CONCLUSIONS: The different results in the literature are due to the individual experience of the author, the very different follow-up methods and the date of follow-up. Therefore, the different results are hard to compare with our study. Nevertheless, Rehbein's anterior resection still could be presented as an adequate and important method to treat Hirschsprung's disease.

[Brain abscess and subdural empyema from the bacteriologic viewpoint]. / Hirnabszess und subdurales Empyem aus bakteriologischer Sicht.

The bacteriology has changed since beginning of antibiotic therapy. Reports of literature and the dates of patients treated in Neurosurgical Clinic of Münster are analyzed. The improvement of microbiological technique leads to the evidence of a greater role of anaerobics and of a smaller part of sterile cultures. The success of therapy depends on the early beginning of specific therapy. The poor prognosis of the sterile inflammations is caused by inefficiency of unspecific therapy.

[Ureter triplication with contralateral ureter duplication]. / Ureter triplex mit kontralateralem Ureter fissus.

A case of ureteral triplication with ectopic ureter and contralateral duplication is reported. The incidence, classification and embryology of triplication are reviewed.

Sexual function after operations for hypospadias according to Ombrédanne.

Patients who were operated upon for hypospadias according to Ombrédanne's method between the years 1963 and 1968 were asked about their sexual experiences and additional information was obtained from their wives or, if unmarried, their girl friends. It is surprising how many open and factual answers to these delicate questions were received. At the time of investigation, the patients were between 19 and 30 years old. Of a total of 142 operated patients, 118 men (83%) replied. Slight chordee of the glans seems to have no influence on sexual experience and sensation. Slight chordee of up to 10 degrees of the shaft of the penis was easier tolerated than a more severe chordee, as we had imagined. This is a preliminary study and it is hoped that further details will be published in the future.

[Carcinoma of the pancreas: palliative surgery (author's transl)]. / Das Pankreas-Carcinom-Palliative Operationen

Among 1146 patients with diseases of the pancreas observed over a 15-year period, there were 234 cases of malignant duodeno-pancreatic tumors. This report concerns the intraoperative diagnostic methods and the various palliative operations available. There was a ratio of 13 males to 10 females, and the average age was 63 years. In 24.3 percent of the patients attempts to establish the diagnosis intraoperatively by puncture or excision were unsuccessful. The cardinal symptom in 71 percent of these cases was icterus. Duodenopancreatectomy was possible only in 21 cases (= 8.9%). In 173 cases (= 75.2% of all patients) a palliative operation was performed.