Progress in pediatric surgery最新文献

A rare complication after ileorectostomy for total aganglionosis of the colon is demonstrated. Eight years after the operation fistulae between rectum and sacrum appeared. Other cases from the literature are mentioned.

A single-chamber pressure probe for rectal electromanometry was developed which seems to be superior to the complicated multichamber systems not only for clinical but also for experimental purposes. Measurements of rectoanal reflex were carried out in 268 cases with abnormal bowel function, in 103 cases following operation for Hirschsprung's disease, and in 61 cases of imperforate anus to assess postoperative continence. Experimental studies were performed in 36 dogs, 27 of which were used for short-term and 9 for long-term studies. The results of clinical and experimental studies are described and discussed, with accompanying literature. From clinical and experimental studies, the neuronal pathways of the rectoanal reflex are schematized. The normal rectoanal reflex is mediated by both the sacral cord and the myenteric neurons. It is concluded that measurements of the anal resting pressure and the rectoanal reflex constitute a valuable method to distinguish between normal and pathological sacral and myenteric innervation.

To clarify the cause of constipation which follows surgery for the supralevator type of disorder associated with the imperforate anus, rectal compliance, percentage maximum static anorectal pressure, and reflex profile were measured by anorectal manometry in 108 normal controls and 42 patients. Patients with constipation had a low percentage anorectal pressure (50%), high rectal compliance, associated with megarectum, and defecation of the staining type with constipation due to a reaction in anorectal motility.

In practical usage, manometry of the rectum and anorectum has proven reliable in providing reproducible measurements; among these are the relaxation reflex, the anorectal pressure profile, and the squeeze pressure produced by active voluntary contraction of the anorectum. In differential diagnosis, there are three major areas of indication: (a) as a screening method in patients with fecal retention to differentiate between constipation and neural disorder; (b) evaluation of continence after surgery for anal agenesis or sphincter replacement, and (c) work-up of residual symptoms after surgery for Hirschsprung's disease. To date, many manometrically obtainable findings have attained chiefly scientific significance.

We report on two children who were admitted with chronic ileus without mechanical obstruction. In the 4-month-old female newborn, high-dose radiation was applied after extirpation of a sympathicoblastoma. Within a few years a metaplasia of the muscle coat of the small intestine developed with a resulting malabsorption syndrome. Although the damaged part of the intestine was resected, the process progressed and the child died. In the second case, a chronic ileus developed at the age of 10 years as a result of fibrosis of the intestinal tract. Repeated laparotomies were performed, and no mechanical obstruction could be found. The most probable diagnosis is a form of scleroderma affecting mainly the alimentary tract without any skin involvement. The patient died in a severe cachexia.

Between 1975 and 1983, 17 neonates with transient functional obstruction of the colon were studied in our surgical department. Five could be successfully treated conservatively with enemas. In the remaining 12 cases colostomy was necessary. In three cases colostomy was performed too late and the patients died. In the other nine cases rectal biopsies and anorectal manometries were performed repeatedly. In spite of clear radiological signs of colonic obstruction such as in Hirschsprung's disease in each case, and identical clinical signs, true aganglionosis could be excluded. Rectoanal manometry 4 months after colostomy showed that the situation had normalized in five cases, but was still pathological in four cases, as in aganglionosis. Of the rectal biopsies, five showed signs of immaturity of ganglionic cells and three were normal. Rectoanal manometry 12-24 months later showed normal reaction in all cases, and of the five cases with immaturity of the ganglionic cells at 4 months one was still pathologic at 12-24 months. In eight of 12 cases the colostomy was closed without relapse of the obstruction, even on long-term follow up. Aganglionosis of the ultrashort type was excluded. In cases of severe transient functional obstruction of the colon in neonates, in which colostomy is necessary, rectoanal manometry and rectal biopsies should be performed as early as possible. Rectoanal manometry, at least, should be done before closure of the colostomy to avoid relapse of the obstruction from closing it to early: the functional disturbance may persist for several months. The term "small left colon syndrome" should be abandoned in favor of "transient functional obstruction,", as the latter describes the clinical condition far better.

The intraluminal pressure of the rectum and anal canal were measured in patients with Hirschsprung's disease before and after Ikeda's Z-shaped anastomosis, and the association of the pressure with postoperative capability of fecal continence was assessed. Radical operation did not alter rectal pressure but did decrease anal-canal pressure. Rhythmical anal contractions increased in frequency until a normal level was attained. The rectoanal relaxation reflex became distinct with time, and 45% of patients eventually attained the reflex after operation. In patients who postoperatively attained satisfactory fecal continence or, at least, only soiling, resting pressure in the anorectum and the frequency of rhythmical anal-canal contractions were similar to those for normal children. The rectoanal relaxation reflex was induced in 58% of the former and 27% of the latter. In patients with postoperative constipation, the intraluminal resting pressure of the anorectum was elevated without the relaxation reflex response. In patients with incontinence, the pressure of the anal canal was low, without a reflex response. These findings indicate that the high and low values of the resting pressure of the anal canal are responsible for constipation and incontinence, respectively, and that the presence of rectoanal relaxation reflex may represent one aspect of a normal defecation function.

Bulk disease is a problem in all the major paediatric sarcomas. It is the pre-eminent problem in parameningeal rhabdomyosarcoma but only a recently recognised problem in limb osteogenic sarcoma (only since the advent of limb-conserving surgery). In all cases where a large bulk of sarcoma threatens to relapse locally, multimodality therapy (surgery, radiotherapy, chemotherapy) stands a better chance of sterilisation than individual modalities of therapy, and such multimodality therapy stands its best chance when it is used early, that is, all three modalities are used at the beginning or shortly after commencement of the treatment course.

The most commonly encountered complications include wound infections, subphrenic collections and bile leaks from the cut liver surface. Stress ulceration of the stomach or duodenum is also not uncommon. Inadvertent bile duct damage has also been reported after major resection in children. The results of surgery for benign lesions are very good and usually depend simply on technical expertise. The results for malignant lesions, however, remain poor. Six series, including our own experience, reported in the last 5 years have shown an overall survival rate of 43% for patients whose tumours were resected for cure. It is difficult to compare results as authors differ in their presentation of results, but details of the series are listed in Table 7. There were no definite prognostic factors to be drawn from these series other than that children with the fibrolamellar variant of hepatocellular carcinoma fared better than those with other hepatomas. It is a sad fact that overall 56% of patients in these series presented with unresectable disease. Mahour et al. described seven patients with unresectable disease who were treated by either chemotherapy alone or in combination with radiotherapy, followed by 'second look' laparotomy and resection. Five of these patients were alive and disease-free for a minimum of 2.5 years after surgery. Thus it is necessary to adopt an aggressive approach to these tumours if one is to see any improvement on the overall figures.