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Background and Purpose: Stimulation electromyography (sEMG) is useful in identifying the sphincter muscle (M) in patients with anorectal malformations (ARMs). This study evaluates the effect of anesthetic agents and M relaxants on sEMG findings., Materials and Methods: Seventeen infants (10 boys and 7 girls) with a mean age of 6.3 months and mean body weight of 6.7 kg were included in a prospective randomized study. Anesthesia was induced by sevoflurane and opioids, and an epidural catheter was inserted caudally. Stimulation electromyography of levator ani M using 14 mA current was used, and latency and amplitude of the evoked compound M action potential (CMAP) were recorded. Patients were randomized into 2 groups. Group A received a local anesthetic epidurally, and sEMG was performed. Administration of the M relaxant and measurement of M response followed. In group B drug administration, sEMG and response measurement were performed after administration of M relaxant., Results: Baseline CMAP was recorded in all patients. Average latency was 4.1 milliseconds, and average amplitude was 0.43 mV. In group A, the average latency was 4.0 milliseconds, and average amplitude was 0.65 mV. After administration of the M relaxant, the CMAP disappeared. In group B, no CMAP was observed immediately after administration of the M relaxant., Conclusion: Administration of the inhalational anesthetic, opioids, and local anesthetic did not influence the M response of M fibers in the levator ani M on sEMG and enables its localization during ARM reconstruction. Nondepolarizing M relaxation completely abolished the response. If M relaxant is necessary, cisatracurium is used. The most suitable method of anesthesia for ARM surgery appears to be inhalational anesthesia supplemented by opioids and epidural analgesia., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Aim of Study: After the treatment of tumors, we often encounter a minimal residual sickness. However, the elimination of these leftover tumor cells is crucial for the patient. In the past years one of the most discussed options for this treatment is Imunotherapy, mainly by Dendritic cells. Dendritic cells are the most efficient cells out of the antigen presenting cell group., Methods and Results: In the first part of the project, we perfected a technique of inducting a tumor on an experimental model. We inducted the tumor by the use of Carcinogenic substances or with the help of the Sarkom line imortalized fibroblasts. Another important part of the project was perfecting the method for the preparation of undeveloped dendritic cells from periphery blood monocytes. After these significant procedures were developed and perfected we moved onto the main part of the study. The Induction of a tumor by the carcenogenic substances Ethylennitrosamin and Phenobarbital was successful only in 20 % of the cases and therefore, was unusable for our experiment. We inducted the tumors with the Sarkom line method. After the application of dendritic cells into the tumor, a decrease in the development of the growth of the tumor was achieved., Conclusion: Imunotherapy using dendritic cells as a basis for treatment is a perspective method for treatment of tumors.

In this study we present the models of preventive and therapeutic vaccination of sarcoma-bearing rats with dendritic cells that present tumour antigens from killed tumour cells. We present the characteristics of dendritic cell-based vaccine and its capacity to induce anti-tumour immune response both in vitro and in vivo. We show that preventive vaccination efficiently prevents tumour growth. On the other hand, vaccination of rats with established tumours did not lead to eradication of the tumours. Despite the induction of a vigorous immune response after administration of dendritic cell-based vaccine and transient decrease in tumour progression, tumours eventually resumed their growth and animals vaccinated with dendritic cells succumbed to cancer. In both settings, preventive and therapeutic, dendritic cell-based vaccination induced a vigorous tumour-specific T-cell response. These results argue for the timing of cancer immunotherapy to the stages of low tumour load. Immunotherapy initiated at the stage of minimal residual disease, after reduction of tumour load by other modalities, will have much better chance to offer a clinical benefit to cancer patients than the immunotherapy at the stage of metastatic disease.

Aim: A technique of approximative anastomosis in witch integrity and continuity of bowel is achieved with limited number of interrupted seromuscular stitches was evaluated in experimental study., Material and Methods: Small bowel anastomosis were performed in twelve rats (Wistar, male) with weight range 197-242 g. An approximative anastomosis in the ileum of six rats was performed with five seromuscular-interrupted sutures only; in the second study group anastomosis was performed with conventional technique of interrupted sutures. The approximative anastomosis was evaluated concerning operating time, anastomotic healing, bursting pressure and adhesions in comparison to the conventional anastomosis. Statistics was calculated with Anova test., Results: All anastomosis in both group healed well without obstruction. The median operating time needed for approximative anastomosis was shorter (31.7 +/- 1.6 minutes versus 35.2 +/- 1.5 minutes, p = 0.002). The strength of approximative anastomosis after 7 days was 249 +/- 39 torr; strength of standard anastomosis was 218 +/- 23 torr (p = 0.118). There were no significant differences in the others evaluated parameters between two study groups., Conclusion: In the animal model presented, the approximative anastomosis shows time saving alternative to standard anastomosis, with the same parameters of anastomotic strength, healing, and adhesions.

Multifocal necrotizing enterocolitis (NEC) may result in extensive bowel necrosis and short bowel syndrome. Authors present case report of premature newborn (BW 1700 g, gestational age 30 w.) where an extensive multisegmental NEC of small and large intestine was found during first explorative laparotomy. Proximal jejunostomy 28 cm beyond ligament of Treitz was performed and the rest of involved intestine was left in situ. After 48 hours multiple small bowel resections were performed leaving 12 cm of small intestine (5 short segments) distal to the jejunostomy. Five approximative anastomoses were performed to restore continuity among these segments and ileocaecal valve. Each of approximative anastomosis was constructed with limited number of 4-6 interrupted stitches and all anastomoses healed without complication. Intestinal continuity between proximal jejunostomy and the reconstructed segment of ileum was reestablished nine weeks later. Total length of small bowel was 50 cm. The patient was discharged at the age of 5 months weighing 4145 g with supplemental pareneteral nutrition. The technique of rapid approximative anastomosis may contribute to save maximal intestinal length in cases with the risk of short bowel syndrome.

Fecal incontinence represents one of consequences of congenital anorectal malformations (CAM) which continues in some patients even after surgeries performed due to malformation and reconstructions. Implantation of artificial bowel sphincter (ABS) is a new solution not proved in daily practice. The aim of the study is to prove the ABS implantation as a feasible solution even in young adults after CAM, which have never been continent, and to verify ABS implantation as a suitable solution., Group of Patients: 11 patients in the age between 18-25 y. were enrolled. 6 patients refused, 5 patients were operated on. 2 ABS implantations were performed. Due to perforation of rectal wall in 1 patient the implantation was postponed. In 2 patients preparative surgeries were performed., Results: 2 patients with ABS implantation and 2 ones after prep surgeries healed primarily. Both ABS are fully functional. Special care is needed to adopt a new defecation habits. Patients with ABS registered increase of QOL score; all pts operated on are both of high motivation and satisfaction.

Hirschsprung's disease (HD) can be associated with the development of neuroendocrine tumours such as medullary thyroid carcinoma (MTC). The RET proto-oncogene is the major gene responsible for both HD and MTC. Mutations in exon 10 (codons 609, 611, 618, 620) were found in patients with co-occurrence of HD and MTC. The aim of the study was to screen the MTC risk in patients with HD. The prospective and retrospective genetic analyses comprised 56 HD patients (41 males, 15 females, aged 0-47). The prospective subgroup of patients consisted of 34 patients (25 boys, 9 girls) operated on between June 2003 and December 2005. The retrospective subgroup comprised 22 patients (16 boys, 6 girls) of 194 patients who were operated on between December 1979 and May 2003, non-systematically chosen preferably for total colonic aganglionosis (TCA). DNAs were isolated from blood and resected segments of aganglionic bowel. The HD patients and nine available family members (2 HD) were tested for RET mutations in exons 10, 11, 13, 14, 15 and 16. Direct double-stranded fluorescent sequencing revealed typical germline heterozygous MTC risk RET mutations in 3/56 (5.4%) female HD patients: Cys609Tyr, Cys620Arg (both exon 10) and Tyr791Phe (exon 13). Two of these patients had TCA and one patient had classical type of HD. One TCA patient developed clinical stage of MTC and underwent total thyroidectomy (TTE). The other two RET positive HD patients (aged 7 and 25 years) are screened for calcitonin level and they are without TTE till now. Two family members (mothers of TCA patients) with detected RET mutation underwent prophylactic TTE with MTC finding. Results showed the benefit of systematic RET mutation screening in HD patients in order to identify the risk of MTC in preclinical stage of the disease in patients with HD and their family members. We recommend to investigate not only exon 10 but also exon 13.

In the last ten years, research has confirmed the role of the RET proto-oncogene in the pathogenesis of thyroid cancer such as medullary thyroid carcinoma (MTC) and papillary thyroid carcinoma (PTC), multiple endocrine neoplasia type 2 (MEN 2) and Hirschsprung's disease that can be associated with MTC or MEN 2. Through the use of molecular genetic testing, we are able to detect gene mutations and the course the disease might take can be predicted, thus enabling us to cure mutation carriers among the high-risk patients can at a very early, clinically asymptomatic stage of the disease; prophylactic total thyreoidectomy in said patients is recommended. At this juncture, there is extensive on-going research on the physiological role played by the RET proto-oncogene on the normal proliferation, differentiation and survival of the cell. Thanks to the new findings there are now possibilities of the theurapeutic use of gene therapy on an RET signaling cascade level in near future.

Aim of Study: To determine a single centre incidence, rate of early and late complications and sequelae and histological characteristics of solitary rectosigmoideal polyps in children., Methods: Retrospective analysis of hospital charts and histological characteristics of patients operated for rectosigmoideal polyps between January 1995 and December 2005. There were 23 children operated on, the study group consists of 20 patients (3 patients were excluded because of insufficient documentation). Demographics, symptomatology, season of first manifestation, localization, histology, postoperative complications and recurrence rate were evaluated., Results: There were 7 boys and 13 girls in the study group (1:1.9), age at manifestation was 2-17 years (average 5.6 y.). The presenting symptom was bleeding in 14 pts, anal prolaps once and combination of both symptoms 5 times. The polyps were localized 3-15 cm from anocutaneous border, on the posterior intestinal wall. Histological examination showed a juvenile polyp in 15 (75%) patients, atypical juvenile polyp and inflammatory polyp in 2 patients each and lymphoid polyp in one case. One patient suffered from a mild bleeding postoperatively. No recurrence was noted., Conclusion: Polyps in children are rare, surgical removal of polyps is a relatively easy operation with minimum complications. Long term follow-up is not necessary. The seasonal occurrence of first symptoms predominantly in autumn and winter months (19 pts) has not been to our knowledge previously published.

The aim of this work was to retrospectively assess clinical and anatomical features of children with Morgagni congenital diaphragmatic hernias. The study group included 5 boys and 3 girls, aged between 5 months and 13 years, at the time of the diagnosis. Morgagni diaphragmatic hernia was diagnosed with non-acute symptoms in 8 children. A retrosternal defect of the diaphragm was left-sided in 7 subjects and bilateral in 1 boy. A hernial sac was found in 6 children. In 3 subjects, the hernial sac contained the liver, in 2 subjects the omentum, in 2 subjects the transverse colon and small intestinal loops and in one child it contained the transverse colon with the omentum. In all cases, the diaphragmatic defect was closed using primary plasty. All subjects healed without complications. A differential diagnosis of congenital Morgagni hernia must be considered in children with unusual respiratory and gastrointestinal symptoms with abnormal x-ray findings on chest examination. A stricture of the congenital Morgagni diaphragmatic hernia is rare, and was not recorded in our group.

Intestinal neuronal dysplasia type B (IND B) is currently defined as a disease of the submucous plexus of the intestine. The aetiology of IND B remains largely obscure. The congenital origin of IND B is supposed; nevertheless, the findings of IND B associated with chronic intestinal obstruction support the notion that this disease could be caused by a reaction of the enteral nervous system to intestinal obstruction or inflammatory disease either in the fetal or the postnatal period. The treatment of IND type B has no unified concept of treatment. The ultimate clinical diagnosis of IND B should be based on a definitive histological diagnosis relating to clinical symptoms, the course of treatment and long-term follow-up of patients with this dysfunction of intestinal motility, despite the fact that no correlations of the clinical picture, radiological investigation and anorectal manometric studies with IND B have been found so far.

Purpose: The RET proto-oncogene is involved in neural crest disorders. Activating germline mutations in the RET proto-oncogene cause the development of familial medullary thyroid carcinoma (FMTC) or medullary thyroid carcinoma (MTC) as a part of multiple endocrine neoplasia type 2 (MEN2) syndrome. Inactivating germline mutations in the RET proto-oncogene are detected in Hirschsprung's disease (HSCR). Only in a very small number of families are these 2 diseases expressed together., Methods: This study presents a novel Czech kindred with FMTC-HSCR phenotype. Two family members (mother and daughter) were tested for RET germline mutations in exons 10, 11, 13, 14, 15, and 16., Results: Direct fluorescent sequencing of genomic DNA revealed a heterozygous mutation in the RET proto-oncogene in exon 10 at codon C609Y in both persons tested. This family was reclassified, thanks to genetic screening from the apparently sporadic MTC-HSCR to FMTC-HSCR., Conclusion: The germline mutation was detected because of the systematic genetic screening of the RET proto-oncogene, which is useful for genetic counseling of potential risk of HSCR and MTC in other family members. This family could be added to the small worldwide cohort of families with MEN2A/FMTC-HSCR.

Aim: The aim of this study is to assess a contemporary treatment potential for necrotizing enterocolitis in newborns with birth weights under 1000 g. METHODOLOGY AND SUBJECTS: This is a retrospective study of clinical and pathological data in a group of 19 newborns with birth weights under 1000 g treated for necrotizing colitis (NEC) by the authors' team from 1999 to 2003. Only newborns with the second and third grade NEC according to Bell were included in the trial group., Results: The trial group included 19 newborns born in the 26th gestation week, on average (ranging from 23- to 31) with a mean birth weight of 711 grams (the range between 460-980 g). Their NEC appeared on the 18th postnatal day, on average (the range between 6-59). Ten newborns were operated in the acute stage of their NEC, nine were treated conservatively. Five infants operated in the acute stage had separational ileostomy conducted, four had a T-drain introduced and one had an abdominal drain introduced. In the conservatively managed group, three infants were consequently operated for intestinal strictures following their NEC, two recovered during the conservative treatment and four exited due to a fast progress of their NEC without surgery. The total mortality rate of the trial group was 42% (8 infants exited)., Conclusion: The NEC treatment in the extremely immature newborns with birth weigts under 1000 g requires complex cooperation of a neonatologist and a paediatric surgeon. The necrotic intestine resections followed by stomic procedures remain standard procedures in unstable newborns with a localised form of the disease.

Background: Alimentary tract duplications (ATDs) are rare congenital anomalies affecting about 1 in 4500 newborns with a male/female ratio of 1.2 : 1. The presence of associated, sometimes complex congenital anomalies can pose a challenge to surgeons treating patients with ATDs. The aim of this work was to study the characteristics of ATDs and associated anomalies in our patients., Methods: Retrospective study. Medical charts of 24 patients (7 males, 17 females) diagnosed with ATD during the years 1990 - 2001 at the Department of Paediatric Surgery, Motol Children's Hospital in Prague, Czech Republic were reviewed., Results: Sixteen patients (67 %) were diagnosed at ages younger than 3 years. Eight cases were foregut duplications, 9 were midgut, and 7 hindgut anomalies. In 13 patients (54 %) associated malformations were documented. Eight patients (33 %) developed postoperative complications, 1 patient died. Complications were more frequent in the group of patients with associated anomalies (53 % vs. 9 %)., Conclusions: Associated congenital malformations are frequently present in patients with ATDs. The complexity of the surgical repair of these anomalies may contribute to higher morbidity and mortality rates. A thorough examination to identify and characterise the presence of ATDs and associated congenital defects would provide appropriate diagnostic and surgical approaches for these patients.

Background: Treatment of the Congenital Diaphragmatic Hernia in neonates with early manifestations has been accompanied with significant mortality. Authors present the treatment protocol employed in such children and results from the period January 1994 to December 2003., Methods and Results: Since January 1994 to December 2003 96 children were cured for the Congenital Diaphragmatic Hernia. Ratio F:M was 36:60. 29 children (30%) were diagnosed prenatally, the remaining newborns were diagnosed on the basis of thoracic X-ray, which was required for the early signs of respiratory insufficiency. The postnatal treatment protocol included circulation-ventilation stabilisation, operation, early extubation and initiation of the peroral nourishment. Altogether 78 (81%) of children were operated after the stabilization using the standard laparotomy method, 5 children (6%) were operated with extra corporal membrane oxygenation, in 20 children (26%) a Goretex patch was used to cover an extensi e defect in the diaphragm. Overall survival was 74%, survival of operated children was 91%, 75% of children with a patch survived, survival of children operated with ECMO was 40%. Relapse occurred in one child (1%) with a patch., Conclusions: Specification of the prenatal diagnosis and collection of patients with CDH into tertiary centres with ECMO possibility can improve prognosis of neonates with the malformation.

The etiology of neuronal intestinal dysplasia remains largely unknown. There is, however, supporting evidence of the existence of Hirschprung's disease or chronic intestinal obstruction associated with neuronal intestinal dysplasia. With the aim of investigating the possible development of neuronal intestinal dysplasia linked to chronic intestinal obstruction, we have examined the enteric nervous system response to long-term obstruction in a rat model. Three different surgical techniques were tested in Wistar male rats. In animals that survived longer than the cutoff chronic intestinal obstruction point (6 weeks), full-thickness biopsies and acetylcholinesterase (AChE), NADH, hematoxylin-eosin, and anti-S100 protein stainings were performed. The results of our model indicate that chronic intestinal obstruction induced different degrees of enteric nervous system dysplasia, including histological features of neuronal intestinal dysplasia. The relationship between chronic intestinal obstruction and anomalies of the enteric nervous system, including neuronal intestinal dysplasia, needs to be further studied.

Background: Survey of surgical techniques, strategy and results of the treatment of patients with Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine in Prague from 1979 to 2004 is presented., Methods and Results: Paper summarises a twenty-five years long retrospective study of medical records of 274 patients who were operated on 1979-2004. 173 (67,3 %) pts had classical rectosigmoid (CRA) and/or long colonic aganglionosis (LCA). 19 (6,4 %) pts had total colonic aganlionosis with small bowel involvement (TCA). 72 (26,3 %) pts suffered from ultra-short rectal aganglionosis (URA). 74 pts with CRA and LCA operated on 1979-1991 underwent Kasai's colorectoplasty. 8 (10,8 %) of them had anastomotic leak, 7 (9,5 %) pts had anastomotic stricture, 2 (2,7 %) pts had postoperative enterocolitis (PEC) and 10 (13,5 %) had chronic constipation. In 6 pts an additional partial sphincteromyectomy of the internal anal sphincter SFME) had to be done. In period 1991-2004 93 pts underwent Swenson's procedure with (SFME). Anastomotic leak occurred in 3 (3,2 %) pts. Anastomotic stricture, PEC and/or chronic constipation were not registered. In 6 pts with anastomotic leak from both groups Soave re-do pull-through was done. In 2003-2004, 10 pts with CRA underwent a transanal resection of aganglionic segment with Swenson's colorectoplasty. One pt developed anal stricture. 3 of 19 pts with TCA operated on in 1979-1990 underwent classic Martin's long side to side ileo-recto-colic anastomosis. In 16 pts of 1991-2004 only short ileo-recto anastomosis according to Kasai and/or Swenson were done. In 93 pts suffering from URA a Lynn's SFME was performed. Three-stage procedure for the last time was used in 3 pts in 1984. Two- and one-stage procedures are still used., Conclusions: A number of postoperative complications decreased, a three-stage procedure was abandonned and the operation of HD is currently performed in newborn. In CRA a TAR replaced the laparatomy and in TCA the short ileo-rectal anastomosis is performed only.

Introduction: The technique of transanal resection (TAR) of aganglionary part in rectosigmoid, published in 1998, has gradually become a standard operation technique in the treatment of Hirschsprung's disease (H.d.). Since the TAR technique for H.d. is not yet generally widespread and has not been used in the Czech Republic yet, the authors intended to share their own experience and early results obtained by this surgical technique. COHORT OF PATIENTS: Seven patients (five boys and two girls) suffering from the classical recto-sigmoid form have been operated on since June 2003. One girl was affected with the Down's syndrome. The age of the patients at the time of operation was between one and 24 months., Surgical Technique: The extent of intestinal resection was determined on the basis of per-operation biopsy. The length of the rectrosigmoid resection was between 25 and 35 cm. The colorectal anastomosis with partial sphincteromyectomy of internal anal sphincter was performed by the technique according to Swenson. The antibiotic prophylaxis was secured by three doses of cefoxitin and one dose of isepamycin., Results: Intestinal passage reestablished 8-12 hours after the surgery, and complete oral intake began on day 3 or 4 after the operation. All patients healed up without complications, the period of hospitalization was seven days on the average. The final diagnosis of H.d. was confirmed by biopsy examination of the dissected portion in all cases., Conclusion: TAR is univocally the method of first choice in the surgery of classical Hirsprung's disease when compared with laparotomy or laparoscopy.

The aim of the work was to evaluate clinical and anatomical features in children with late manifestations of inborn diaphragma hernia after the newborn period. The group included 11 boys and 7 girls at the age of two to 78 months during the time of diagnosis. The diaphragma hernia was diagnosed in 15 children during non-acute manifestations and in three children affected by emergencies. A posterior-lateral defect of diaphragma was on the left side in 11 children and in 7 children on the left. A hernia sac was found in 11 children. Organs protruding into thorax included intestinal loops in 12 children, liver in 7 subjects, spleen in 6 individuals, stomach in five and kidney, pancreas and omentum in one each. The defect in diaphragma was occluded by a primary plasty in 17 children, while a patch from Goretex was used once. An intestinal strangulation required resection of necrotic part of intestine, but the boy died one month later for a multi-organ failure despite long-term resuscitation care. The other children were cured up without complications. The inborn diaphragma hernia should be considered in differential diagnosis of every child with unusual respiratory or gastrointestinal symptoms and abnormal X-ray picture of thorax. Acute strangulation of inborn diaphragma hernia after the newborn period is a life-threatening disease, where the gastrointestinal obstruction is combined with respiratory and circulation failure.

The objective of the present work is a review of contemporary therapeutic possibilities of neonates with serious developmental defects. Neonatal surgery of congenital developmental defects was in recent decades favourably influenced by the development of neonatology and prenatal diagnosis. Nowadays prenatally diagnosed congenital anomaly is not a primary indication for termination of pregnancy but for comprehensive examination and consultation in a specialized centre. The definite decision regarding continuation of pregnancy or its termination is up to fully informed parents. In the majority neonatal surgery is not surgery of urgent operations, adequately stabilized neonates are operated while ensuring comprehensive extrasurgical care. The prognosis as regards survival and quality of life is favourable for the majority of isolated congenital defects. During the period 1994-2001 at the authors' departments a 73% survival of neonates with an isolated diaphragmatic hernia was recorded, 87% with omphalocele, 93% with gastroschisis, 90% with oesophageal atresia, 97% with anorectal atresia and 100% with intestinal atresia. With declining mortality, in the foreground of interest are problems of early definitive correction of long-term results and quality of life. With regard to the low incidence of different congenital anomalies and the contemporary birth rate in the Czech Republic its is justified to concentrate selected defects in centres which are able to provide comprehensive prenatal and early postnatal treatment.

Background: Evaluation of faecal continence, education and occupation of patients operated during childhood on account of Hirschsprung's disease by Kasai's colo(ileo)rectoplasty., Material and Methods: Analysis of data from questionnaire, ambulatory check-up examinations and case-records of 46 patients of 109 operated in 1979-1995., Results: Of 46 patients (36 men, 10 women) aged 18 to 35 years (mean age 22.5 years) 42 patients (33 M, 9 F) had the classical or long type of HD, 4 patients (3 M, 1 F) suffered from aganglionosis of the entire colon (TCA). The follow-up period after operation was 8 to 24 years (on average 13.5 years) 23 patients (50%) were quite free from complaints, 23 patients (50%) have occasional complaints: constipation 8 (14.4%), diarrhoea 5 (10.9%), soiling 20 (43.5%), abdominal pain 16 (34.8%). One patient has pain in the rectum, one female patient painful sexual intercourse, 4 (8.7%) suffer from meteorism. 6(13%) patients use laxatives, 8 (17.4%) enemas. Four (8.7%) patients were reoperated on account of residual aganglionosis. Seven (15.2%) patients had a partial sphincteromyectomy of the internal anal sphincter. Two patients had ileus with adhesion, one patient resection of a urethral stricture, one female patient with TCA total thyroidectomy on account of a medullary thyroid carcinoma. Eight (16.3%) patients have elementary education, 14 (30.4%) completed an apprenticeship, 22 (47.8%) have secondary education, 2 (4.3%) have university education. Forty four (95.6%) patients work full time., Conclusion: Although occasional soiling is reported by 43.5% patients, all patients evaluate their situation as good or adequate.

During the period between 1980-2000 at the authors department 5114 children with acute appendicitis were operated (on average 245 per year). From this period the year 1999 was selected when 160 children were operated on account of acute appendicitis. Thirty-eight children had an advanced finding--gangrenous appendicitis or an abscess. Gangrenous appendicitis or empyema of the appendix without perforation were recorded in 16 patients (10%), gangrenous appendicitis with perforation in 9 patients (5.6%), a periappendicular abscess in 13 (8.1%), phlegmonous appendicitis was recorded in 108 patients (67.5%) and in 14 patients the histological examination revealed mesenterial lymphadenitis (8.7%). The authors demonstrate on selected case-histories the problem of appendicitis not recognized in time and appendicitis with an atypical course. They draw attention to the uncommon clinical course in 5 patients where at first a tumour of the upper mediastinum, tumour of the abdominal cavity, twice diarrhoeal disease and a tumour in the lesser pelvis was suspected. The authors emphasize that even at a time when special examinations are available (ultrasonography, computed tomgraphy) it is important to make repeated clinical examinations of the patients at least in the course of three days.

A girl suffering from chronic constipation and abdominal distension from her first year of life underwent internal anal sphincter myectomies at 5 and 7 years of age without resolution of her symptoms. At the age of 8, an ileostomy was performed because of excessive colonic dilation and hypomotility. Biopsies from the colon and distal ileum showed intestinal neuronal dysplasia TYPE B (INDB) with hypoganglionic areas. Colectomy and ileorectal anastomosis were done at the age of 10. Three years later, however, an ileostomy was re-established because of recurrent episodes of pseudo-obstruction. In the hope of improving intestinal motility, the dilated small intestine was tapered over its entire length of 3.6 meters. Histological findings still demonstrated oligoneuronal hypoganglionosis and INDB all along the resected strip of bowel wall. After 6 months, the stoma was closed. At the age of 15 years, tapering of the distal 80 cm of the ileum was repeated in combination with cholecystectomy for cholecystolithiasis. Intestinal transit time decreased from 55 hours before the first to 18 hours after the second tapering procedure. Now, 7 years after the last operation, the patient passes 3 - 4 soft stools daily, is physically active, on a normal diet and not on any regular medication.

The authors present their experience with the reconstruction of the diaphragm with a synthetic polytetrafluoroethylene patch in a group of 66 neonates with a congenital diaphragmatic hernia. After preoperative stabilization 53 neonates (80%) were operated, simple reconstruction of the diaphragm was made in 39 neonates (74%), reconstruction of the diaphragm with a polytetrafluoroethylene patch was indicated in 14 patients (26%). Nine patients recovered after reconstruction of the diaphragm by a synthetic patch without complications. The only relapse of diaphragmatic hernia when using a patch was recorded in a neonate with a bilateral diaphragmatic hernia, sternal cleft and omphalocele. After simple reconstruction of the diaphragm there was no relapse of hernia. The total mortality in the group was 27% (18 neonates). After surgery five neonates died. Two of them died of serious haemorrhagic complications during extracorporeal membrane oxygenation and three died after operations performed at borderline oxygenation and ventilation values and subsequent deterioration of the general condition. In four of them a polytetrafluoroethylene patch (GORE-TEX) was used. Reconstruction of the diaphragm by a polytetrafluoroethylene patch is a suitable surgical method in the treatment of neonates with a congenital diaphragmatic hernia. Indication for the use of a patch is agenesis of the diaphragm and major diaphragmatic defects with hypoplastic borders. The use of a non-absorbable synthetic patch is from the aspect of the long-term effect on growth and the development of skeletal deformities equivocal and will call for further detailed studies.

The aim of the paper is to compare different techniques of correction of hypospadias with the author's own group of patients and to compare the results with those achieved at other departments. Between 1992-1999 at the Department of Paediatric Surgery, Teaching Hospital Motol 348 operations were made in 219 boys. A plastic operation with a distal shift of the urethra a and conization of the glans in 112 patients predominated. Other methods included in 39 patients the creation of a ventral circumference of the urethra from the inner laver of the prepuce while preserving the urethral plate ("onlay island flap") and a parameatally everted flap according to Mathieu in 11 patients. A plastic operation by complete tubulization of the inner laver of the prepuce was used in 19 patients, in 2 boys the method of Duplay-Byars tubulization of the urethral plate. In one patient the urethra was replaced by a free flap as described by Nove Josserand. Another 129 operations included formerly used and now abandoned two-stage operations to straighten the penis, operations to reconstruct the prepuce and operations on account of complications. The latter comprised 16.4% fistulae, 9.58% dehiscences and 1.36% strictures.

Feminizing genitoplasty (FGP) involves modification of a hypertrophic clitoris, vaginoplasty, and modelling of the labia. FGP is performed during childhood and usually in one stage. In 1996-1999 30 patients with intersex were operated on. 25 girls with female pseudohermaphroditism caused by congenital adrenal hyperplasia (CAH), 3 patients with mixed gonadal dysgenesis (MGD), 1 patient with male pseudohermaphroditism and one female patient with true hermaphroditism (HFV). Seventeen patients were between the ages of 3 and 6 years, 13 patients (all female with CAH) were 13 to 21 years old. One-stage FGP, i.e. partial resection of the clitoris and vaginoplasty (VP) was performed in 11 patients. A two-stage operation, i.e. partial resection of the clitoris and subsequent vaginoplasty, was done in 3 patients (all with VHN with a high-orifice vagina). Vaginoplasty after excision of the clitoris was made in 13 patients (all with CAH, 5 of them with a high-orifice vagina). Three female patients (2 VHN, 1 HFV) had partial resection of the clitoris and wait to have VP. Fourteen patients with partial resection of the clitoris healed p.p. Healing p.s. was recorded in 5/27 patients with vaginoplasty (all CAH). Three of them were older, menstruating and obese. In 4/14 patients after partial resection of the clitoris both modelling and fixation of the glans clitoridis was necessary. Urinary incontinence was not recorded. Stricture of the vaginal orifice was found in one 6-year-old patient with CAH after reconstruction of a high-orifice vagina. Thirteen older patients have a normal vaginal orifice, two of them have sexual intercourse. FGP can be performed already at toddler age, the cosmetic effect is favourable, the glans clitoridis is preserved and the psychosexual development of patients is not impaired. FGP can be used also in adults, severely virilized patients.

Posterior sagittal anorectoplasty is a marked change in treatment of anorectal malformations and differs fundamentally from previous techniques. In 1991-1996 86 patients (51 boys and 35 girls) aged 1 day to 22 years were operated. A primary operation was performed in 65 patients, a secondary one in 21 patients. In primary operations posterior sagittal anorectoplasty was used in 50 patients, in 3 cloacal malformations it was extended by reconstruction of the vagina and urethra. Fifteen patients were operated by other techniques. Posterior sagittal anorecto (vaginourethro) plasty was used in all 21 reoperated patients. Of 65 patients with primary operations one patient died from aspiration bronchopneumonia four years after operation. The patient had also an operation on account of oesophageal atresia. Three patients were reoperated. The continence of patients after primary operations is satisfactory, 5-6 points on Kelly's scale. In 19/21 reoperated patients the continence is 3-5 points according to Kelly. In two patients no substantial changes were recorded, one was reoperated twice. As compared with pull-through techniques the posterior surgical method of anorecto (vaginourethro) plasty is excellent. It cannot be used however without adequate experience.

Extensive intestinal resections in inborn intestinal atresias are the second most frequent cause of the short gut syndrome. Because treatment of this condition is so far minimal, prevention is of fundamental importance. One possible approach is tapering of the gut, i.e. longitudinal antimesenterial resection of the gut. At the Clinic of Paediatric Surgery in 1991-1995 30 patients with inborn atresias of the gut were operated (17 atresias of the duodenum, 11 atresias of the small intestine, 2 atresias of the large intestine). Six patients (20%) died. In 2 patients (one girl with atresia of the colon and one boy with atresia of the jejunum) developed dilatation of the gut orally from the site of resection of the atresia and a chronic subileous condition. Instead of resection of the dilated portion the gut was modelled by tapering. In both children the passage improved and the children thrive. Based on data in the literature and their own experience the authors assume that tapering of the gut should supplement primarily high jejunal atresia, apple peel syndrome and extensive dilatation of the jejunum. Tapering cannot be used above the aganglionic portion of the intestine.

The objective of the presented paper is to define the characteristics of colo(ileo)rectoanastomosis for the treatment of Hirschsprung's disease (H. d.) and other congenital malformations in the innervation of the distal gut (CMDI). During 1979-1994 at the Clinic of Paediatric Surgery of the Second Medical Faculty in Prague-Motol 137 patients (100 boys and 37 girls), aged 5 months to 18 years with H. d. and CMDI were operated. In 124 patients Kasai's colorectoplasty was used, in 40 of them supplemented by partial sphincteromyectomy of the internal sphincter of the anus (SPME) and Swenson's transanal colorectal anastomosis. In 10 patients with total aganglionosis of the colon (TCA) in three instances ileorectoplasty and Martin's anastomosis was used, in seven instances only ileorectoplasty. In three patients the authors used Soave's endorectal pull-through. 85 patients (62.1%) had no postoperative complications. Early infection was recorded in 12 patients (8.6%), dehiscence of the surgical wound in seven patients (5.1%). Dehiscence of the colo(ileo)rectorectal anastomosis occurred in 13 patients (9.5%), stricture in 10 patients (7.2%). Postoperative obstruction of the gut was recorded in 7 (5.1%) patients, postoperative enterocolitis in three patients (2.2%). There were no deaths. Regular opening of the bowels after 1-2-day intervals was achieved in 110 patients (84.1%). Patients after surgery of TCA have on average 2-5 stools per day. Sixteen patients developed chronic constipation and subileous conditions. Incontinence of faeces was found in two patients with Down's disease. Colo(ileo)rectoplasty in H. d. and other CMDI should meet the following conditions: maximal resection of the affected portion--creation of a satisfactory anastomosis with a minimal occurrence of strictures and dehiscences--preservation of satisfactory continence--elimination of anorectal sphincteroachalasia. Modified Kasai's rectoplasty or Swenson's procedure meet these conditions. In case of TCA a 10-15 cm ileorectocolic anastomosis is quite sufficient. For reoperations of strictures and inflammatory complications after previous colo(ileo)rectoplasty Soave's technique is probably the best choice.

A reaction for NADH tetrazolium reductase was used to demonstrate the presence of ganglion cells in myenteric plexus during dysganglionic bowel operations in 24 patients. The advantages of this method are the quick preparation of an incubation medium, a short incubation time and the good demonstration of ganglion cells. It was possible to determine the extent of the aganglionic and prominently hypoganglionic parts of the bowel and in this way achieve improved results. The NADH tetrazolium reductase reaction can be recommended for the perioperative biopsy of a dysganglionic bowel.

The authors present their experience with resection of the liver in children. In 1986-1993 they performed a total of 34 major resections of the liver. The mean age of the operated children was 4 years and 5 months, the youngest patient was operated at the age of 4 days and the oldest one at the age of 17 years. The group was divided into two time periods which are compared, because in 1990 the authors changed their surgical tactics and technique and also the procedure used for general anaesthesia. During the first period/1986-1989/they made 12 resections of the liver and during the second period/1990-1993/22 resections. The two groups of patients are compared as regards mortality during operation, early and late deaths. In the first period the mortality was 25%, in the second period no death occurred during operation. Early deaths were recorded during the first period in 25%, during the second period in 4.5%. Patient survival during the first period is 33% and during the second period 86.5%. The authors describe the technique of resection of the liver they use at present with emphasis on isolation of the vena cava inferior with the possibility of easier control of haemorrhage and the use of an ultrasonic aspirator/CUSA/and laser during resection proper which makes easier identification of hepatic structures and control of blood losses on operation possible. In malignant tumours the radicality of surgery increased as manifested on patient survival. From the first period 15% patients survive, from the second period 83% without signs of relapse.

The authors examined peroperatively 41 excisions from the muscularis externa of the large bowel in 20 patients operated on account of intestinal dysganglionosis. For visualization of the ganglion cells the reaction for NADH tetrazolium reductase was used. The advantage of this method is that the incubation medium can be very rapidly prepared from a stock solution and that the incubation period is short. The visualization of ganglion cells is by contrast. In all instances it was possible to assess the length of the aganglionic and markedly hypoganglionic portion of the gut and make thus the result of the operation safer.

A method for diagnosis of isolated tracheo-oesophageal fistula by oesophageal manometry is described. It consists of pull-through registration of intraluminal pressures. An abrupt pressure rise in the area of the fistula during inspiration is pathological. In principle, this method consists of the graphical tracing of the original Koops test which we have slightly modified. The investigation is non-invasive and accurate in comparison with roentgenological and/or endoscopical methods.