Progress in pediatric surgery最新文献

The cloacal anomaly is characterised by the persistence of a common channel draining the urinary, genital and alimentary tracts via a single orifice. It results from abnormal compartmentalisation of features that are normal in the primitive female embryo. Abnormal embryology and cloacal anatomy are described in detail. Cloacal abnormalities are usually diagnosed promptly in the neonatal period. Management can be divided into three phases: (1) investigating and defining the anatomy, (2) neonatal intervention with relief of obstruction and (3) definitive surgical reconstruction. Successful management of the child with a cloacal abnormality remains one of the greatest challenges to the pediatric surgeon.

Recent investigations and reports on late results indicate that vaginal orgasm is more the exception than the rule, so that, for a woman, preservation of clitoral sensitivity is essential to a satisfying sexual life. All techniques involving total clitoridectomy, plastic imitations, or displacement of the clitoris under the symphysis must therefore be discarded. Even if plication or trapping of an enlarged clitoral shaft under the mons veneris can be regarded as sensitivity-maintaining procedures, they nevertheless do not yield satisfactory results, since painful sensations or a feeling of pressure may occur during erection. Hence, reduction-planess should use techniques which shorten the erectile parts of the clitoris and reduce its size, while still maintaining sensitivity. Good cosmetic and tactile results may be achieved by means of selective excision of the corpora cavernosa and lateral clitoral excisions. Reconstruction of the labia minora out of clitoral shaft skin is combined with separate creation of a neo-preputium clitoridis. Vaginal enlargement plasties have always been problematic, since shrinking particularly of the vaginal introitus, occurs in up to 25% of patients who undergo this operation. However, a sufficiently large pediculated perineal skin flap inserted into the "defect" of the posterior vaginal wall provides sufficient width of the vaginal introitus and canal. Partial vaginal aplasia, with the vagina opening into a urogenital sinus near the bladder, calls for additional abdominal mobilisation. For psychological reasons, vaginal dilatations are not to be recommended. If necessary, a second vaginal enlargement plasty should instead be performed later; this may be carried out without problems before puberty. To avoid the disadvantage of a dry skin flap which does not assimilate to normal vaginal mucosa even after many years and with oestrogen treatment, mobilisation of the posterior vaginal wall with displacement of real vaginal mucosa towards the perineum can be carried out. However, one-stage reconstruction of clitoris, vulva and vagina during early childhood is preferable in every case, in order to avoid the psychological damage which can undoubtedly otherwise be caused. An exception is the late onset form of congenital adrenal hyperplasia.

Every second patient with a solitary kidney suffers from renal disease. This accumulation of renal diseases of varying origin makes special care for these children necessary. The quality of diagnostic methods is decisive for the choice of therapy. The advantages of primary ultrasound diagnosis and sonographical function tests are described. Sonographical differentiation of disturbances of the urinary transport is possible by means of forced-diuresis ultrasonography, whereas a vesico-ureteral reflux can be detected by means of voiding sonocystography. The value of ultrasound for primary diagnosis, assessment of therapeutic course and postoperative long-term follow-up is discussed.

Surgery, radiotherapy and chemotherapy are currently the basis of multimodal treatment of Wilms' tumor. Surgery plays the central role in the management of this tumour and will cure 25% of patients if employed alone. Surgical mortality has been reduced to 1.5% at major centres and the most dangerous intra-operative complication is a venacaval tumour embolising into the pulmonary artery. Patients found to be initially inoperable who then have local and systemic therapy, followed by successful secondary surgery, have a reduced survival rate. Definite statements on bilateral tumours are difficult to make. The most fundamental determinants of survival are the histological characteristics of the tumour and the stage of the disease at presentation. Just as the quality of surgery has improved, so have radiotherapy techniques been refined. However, radiotherapy has been replaced by chemotherapy in many instances. The advent of chemotherapy has added to clinical success with a further improvement of survival figures by 25%. Chemotherapy is most effective in controlling micrometastases. However, it must be remembered that the treatment is toxic and needs skillful handling and modification.

The purpose of this study is to analyze the results of anorectal manometry and to evaluate the merits and disadvantages of this technique for the diagnosis of Hirschsprung's disease. Studies were performed in 268 patients with constipation, including 95 cases of Hirschsprung's disease. It is concluded from the results that Hirschsprung's disease can be confidently diagnosed by manometric studies. If the studies are performed carefully with a suitable probe, reliability is over 95%. Manometry is the most useful method to differentiate Hirschsprung's disease from other conditions, such as extremely short segment aganglionosis, colonic stenosis, and idiopathic megacolon.

Functional results after surgical correction of anorectal malformations were assessed on a clinical basis using the Kelly score and by manometric study. In all, 65 patients, aged 5-28 years, were interviewed personally, and 51 of these 65 had manometric studies to evaluate postoperative continence. The manometric study was also performed on 45 normal children as control group. Continent patients characteristically had a marked high-pressure zone, as did the normal subjects. On the other hand, in the patients with fair or poor results, the anorectal pressure profile had no marked high-pressure zone in the anal canal. The presence of normal anal pressure at rest as well as adequate anorectal pressure difference was found to correlate well with continence. In the patients with perineoplasty, the anorectal reflex correlated well with continence, but not in patients treated by abdominoperineal rectoplasty.

Three types of neuronal intestinal dysplasia (type A, type B, and combination with Hirschsprung's disease) can be distinguished. Functional assessment of the affected bowel segments can be achieved by functional colonic ultrasonography, thus providing exact parameters for further therapeutical procedure. The technique is described. Ten children with neuronal intestinal dysplasia in whom functional colonic ultrasonography was employed and results of their follow-up examinations are reported.

The problem of second primary tumours is likely to increase in magnitude as the number of long-term survivors of childhood cancer grows and treatment protocols are intensified. Children with an underlying genetic disease appear to be at particular risk of developing a second tumour. While most cases of second malignancy appear to be associated with either radiotherapy or chemotherapy, a small proportion of patients have no identifiable risk factor. In these children, unrecognised predisposition or, indeed, chance may play a role. It would seem to be important to identify factors such as genetic susceptibility and specific modalities of therapy, including ionising radiation and alkylating agents, which may contribute to the development of second tumours, because awareness of the risk factors may make it possible to modify treatment programmes and thereby minimise the risk of second neoplasms. Long-term surveillance of patients treated for cancer during childhood is recommended so that the problem of second malignancies can be monitored.

Thirty-three patients, ranging in age from newborn to 12 years, with fibrous tissue tumours, were treated at the Hospital for Sick Children, Great Ormond Street, London, from 1970 to 1984. Seven infants presented with tumours at birth. In 42% of cases, the tumour was noted during the 1st year of life. The anatomical distribution of the lesions was: upper extremity 6, lower extremity 5, head and neck 10, trunk 6, thoracic cavity 2 (heart 1), abdominal cavity 4 (pelvis, bladder, small bowel mesentery, and canal). Three patients had multiple tumours. Seven of the tumours were classified as sarcomas. Complete excision was possible in 28 patients, following initial biopsy in six patients of whom three were treated preoperatively with radiotherapy and/or chemotherapy. There were two deaths, one in an infant with a huge resectable pelvic tumour and the other in a child with cardiac fibroma. Recurrences occurred in 14 patients (48%), in nine of whom the recurrences were multiple. The mean time interval to recurrence was 13.8 months.

Two children with stage IV neuroblastoma died from severe reactions of the bowel due to radio-chemotherapy. This led to the suggestion of protecting the bowel by implantation of a silastic balloon to push the bowel away from the treatment volume. This procedure was tried in two children with stage IV neuroblastoma and resulted in excellent tolerance of the high-dose radiotherapy.