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Posterior sagittal approach is very useful for the correction of anorectal malformations, but it can be used also for the treatment of pelvic or perineal masses. The authors present 3 patients affected one by a perineal hamartoma, one by a rectal duplication and one by a retrorectal cystic teratoma. All the patients were treated following the PSARP technique, using the Penã's bipolar electro-stimulator just in order to respect all the muscles of the anorectal sphincters. Using this procedure the authors were able to preserve the full anorectal continence in all the 3 patients.

Ten patients with anorectal malformations were studied by preoperative CT: well developed sphincteric muscular structures (puborectal sling of levator ani, muscular striated complex and external sphincter) were present in six patients. In three patients muscular structures were poorly developed (case 2a with rectovaginal fistula, case 5a with rectocloacal fistula and case 7a with prostatic fistula). In one case with bladder fistula, the sphincteric musculature was not identified at all. Eleven patients were postoperative studied by pelvic CT: Two of 11 cases had a good sphincteric control. The other nine patients were incontinent. In three of the nine incontinent patients, the colon was appropriately place, but puborectal muscle and sphincteric complex were hypoplastic. In the other six cases CT showed a pulled through colon out of the sphincteric structures. Four of them presented major bony sacral abnormalities and/or lesion of the sphincteric muscle structures. The remaining two patients with an evident pulled-through colon out of a normally developed sphincteric region and without sacral anomalies, were selected for a secondary PSARP procedure. In one of these two patients the operation brought about a complete resolution of the fecal incontinence. The pelvic CT is a useful examination in the anorectal malformation for the preoperative prognostic evaluation of continence, while postoperative CT is essential in selecting those incontinent patients who need a secondary PSARP for the treatment of fecal incontinence.

The Authors present the cases of cholangitis in childhood observed in the Institute of Infectious diseases of Genoa from 1973 to 1982. Cholangitis rarely starts by alone, whereas it appears usually in association with hepatic underlying disease. In childhood the symptomatological triad (fever, jaundice, pain) is infrequent: only jaundice is a common sign of disease. Polymicrobial aetiology of cholangitis is very usual, but Streptococci and E. coli are the aetiological agents most frequently observed; sometimes also anaerobic organsimee, fungi and protozoa cause cholangitis.

In spite of great progress in surgical treatment of anorectal malformations, fecal incontinence is still, in variable degrees, a frequent and unpleasant postsurgical sequela. The most frequent causes of incontinence are: i) the incorrect placement of the pulled-through colon in the levator ani and sphincteric muscular complex during abdominoperineal surgical procedures; ii) the poor development of sphincteric musculature; iii) the associated sacral anomalies. Postoperative CT helps to evaluate all the above-mentioned conditions, in view of possible new surgical procedure for improving continence (besides postoperative CT can help in choosing the more suitable surgical technique). Nine patients, aged 3 to 13 years, (2 with good continence and 7 with various degrees of incontinence), were studied with pelvic postoperative CT. In the cases (2) with good continence the CT picture was: good development of sphincteric musculature and neo-anorectum correctly placed into sphincteric musculature; in the cases (3) with low degree of continence: neoanorectum correctly placed, but hypoplastic puborectal muscle; in the cases (4) with complete incontinence, neoanorectum incorrectly placed and poor development of sphincteric musculature. A further posterior sagittal anorectoplasty (according Peña) is only suitable in the incontinence cases with: i) neoanorectum seriously misplaced; ii) good development of sphincteric musculature; iii) absence of sacral anomalies. Postoperative CT is a valid mean for demonstrating all the above-mentioned conditions and for choosing the best surgical technique in each case.

In spite of the progress in the field of surgical treatment of anorectal malformations, faecal incontinence is, in variable degrees, still an unpleasant and frequent postoperative sequela. Postoperative CT demonstrate the location of the pulled-through intestine, including whether it had been correctly placed through the levator ani and in the spincteric muscular complex. In our nine patients we discovered a correlation between the CT findings and the clinical picture. The cases of incontinence whether associated with sacral anomalies or not were characterized either by marked hypoplasia of musculature or by the pull-through having missed the sphincteric musculature. CT scans could be of use in planning further surgery.

The radiological findings pathognomonic of Hirschsprung's disease have been known for quite a long time. However, in addition to those diseases characterized by rectum or rectum-sigmoid aganglionosis (as in the classic form of Hirschsprung's disease), in pediatric pathology of surgical interest severe affections exist characterized by aganglionosis extending from the rectum to the entire colon, and exceptionally as far as the small bowel (ultra-long Hirschsprung's disease). The authors report on the results they obtained in radiological studies of 6 cases of total colonic aganglionosis by means of the current radiological procedures, i.e. plain abdominal films and barium enema. As for total colonic aganglionosis, no diagnostic radiological parameters have been assessed yet. The authors compare their results with those taken from international literature: microcolon with reflux into mega-ileum (2 out of 6 cases) appears to be the most suggestive X-ray finding for total colonic aganglionosis. Finally, the role is discussed of radiological studies in the diagnostic approach to total colonic aganglionosis. The authors conclude that, even though radiological investigations are indispensable, they are often not conclusive. Therefore, an accurate evaluation of the proximal extension of aganglionosis can be made only at surgery.

Six children who had benign cutaneous vascular lesions characterized by papillary or solid proliferation of the endothelial cells were seen in a 16-year period. In five of them, the lesions had the histologic features of the entity originally described by Masson and recently reported by Clearkin and Enzinger as intravascular papillary endothelial hyperplasia. In one of the five, the lesion appeared in the pure form within large vascular spaces, whereas in the remaining four it appeared as a very limited focal change in a hematoma. The authors agree with others that this lesion represents a peculiar variant of an organizing thrombus. The histologic features of the lesion in the sixth patient were compatible with the so-called "intravenous atypical vascular proliferation," another pseudomalignant vascular disorder recently described by Rosai and Akerman.

The authors describe 6 cases of Apple Peel Small Bowel observed in the last ten year experience in Institute G. Gaslini of Genoa, Italy. They make a precision on early diagnosis and on surgical treatment, which is original, and also on post-operatory medical treatment. The importance of making an optimal nutritional support with total and partial parenteral nutrition for long time is of great significance to have a better outcome and psycho-physical development in such patients.

The authors present the surgical technique for the correction of Hirschsprung disease following SOAVE's original description. A series of 270 cases observed in 20 years at the Pediatric Surgery Department of the "Instituto Giannina Gaslini" in Genova, Italy, are examined. These cases, all treated by surgery, were followed up in long term for more than one year both clinically and radiologically. Additionally 73 cases treated from 1977 on were also followed up with the aid of ano-rectal electro-manometry. The most recent diagnostic aspects are discussed, such as ano-manometry, and some conclusions are drawn about long term results.

A protocol has been applied to all our patients consulting for nocturnal enuresis who also presented daytime wetting or urge to void, to rule out obstructive uropathies. Our protocol includes voiding cystourethrogram, IVP, cystometry and endoscopy. 64 females and 46 males, 4 to 14 year-old, were studied between 1980 and 1983. Obstructive urethral uropathy was ruled out in 37 females (58%) and 32 males (69%). The most common treatments in these patients were endoscopic resection (Bugbee) of posterior urethral valves in boys and resection of the distal ring in girls. All other associated diseases or malformations were treated according to their etiology. Results, analyzed through a questionnaire sent to parents, were good in 77% of the patients treated for obstruction. In conclusion we think that daytime wetting and urge to void are very frequently the emerging symptoms of a hidden uropathy, in most cases obstructing proximal or distal urethra. Surgical treatment can lead to a good cure rate.

Intraoperative biopsies are required to confirm diagnosis of Hirschsprung's disease and to determine exact extent of aganglionism. Authors report a new method for enzymo-histochemical intraoperative evaluation of aganglionic segment. Activity of non specific esterase (following Davis and Orstein technique, 1959) has been used to demonstrate presence or absence of intramural ganglion cells in sero-muscular intestinal biopsies, which had been taken at various levels in 34 patients with preoperative diagnosis of Hirschsprung's disease. Authors consider histochemical technique as the most reliable, quick, simple and low-cost method for intraoperative evaluation of the length of aganglionic segment and, of course, to confirm diagnosis. Alpha-naphthyl-esterase is also of great help in the study of so-called pseudo-Hirschsprung.

The authors describe a case of adenocarcinoma of the rectum in a 10-yr-old boy. The clinical picture, the surgical technique, and the histology are described, an reference is made to the small number of similar cases appearing in the world literature.

According to their experience and to a review of the most important papers in the field, the Authors examine the clinical, morphological, diagnostical and therapeutical aspects of congenital hepatic fibrosis. The initial sign is almost always hepatosplenomegaly and diagnosis can be achieved by means of clinical, anamnestic, histological and ecotomographic findings. Relationships among congenital hepatic fibrosis, infantile polycystic disease of the kidney and liver and Caroli's disease are stressed.

The anatomical patterns of anorectal malformations have so far been studied according to the principles which inspire Peña's technique for the surgical treatment of anorectal anomalies. Thus, the diagnostic study of anorectal malformations has by the authors been considered a work of classification, but of identification. Among the diagnostic procedures in use in our Institute, preoperative CT of pelvis is performed to assess the presence and to define the development of muscular sphincteric structures towards prognostic evaluation of continence, the major long-term goal. Seven patients, aged 1-30 months, with anorectal anomalies were studied with preoperative CT of the pelvis. CT scans showed well-developed sphincteric muscles in 3 patients (2 with rectoureteral and 1 with rectovestibular fistulas), poorly developed muscular structures in 3 patients (with rectovaginal, cloacal and prostatic fistulas), absence of muscular structures in 1 case (with rectovesical fistula). CT findings were always confirmed at surgery, except for the case with rectovesical malformation where CT scans did not allow to identify the thin sphincteric musculature. The authors believe preoperative CT of the pelvis, together with other clinical and radiological examinations, to be a valid mean in the prognostic evaluation of continence.