[Nephroblastoma -  30-years period of its treatment in the University Hospital Motol, Prague]. / Nefroblastom -  30 let lécby ve Fakultní nemocnici v Motole.

INTRODUCTION: Nephroblastoma (Wilms tumor - WT) is the most common solid tumor of kidney in children. We present treatment development of WT at the Department of Pediatric Hematology and Oncology, Charles University in Prague, 2nd Faculty of Medicine and University Hospital Motol (KDHO) in the Czech Republic over 30 years. Patients that were treated prior to access to the International Society of Pediatric Oncology (SIOP) protocols are considered to be the historical group, then we have patients treated according to SIOP 9, SIOP 93-01 and SIOP 2001 protocols as full participants of SIOP studies. PATIENTS AND METHODS: Between January 1980 and April 2009, we treated 330 patients with WT at KDHO: 91 patients in historical group (1980-1988), 94 pts in SIOP 9 (1988-1993), 80 pts in SIOP 93-01 (1994-2001) and 65 pts in SIOP 2001 (2002-2009). Overall survival (OS) and event-free survival (EFS) were analyzed by Kaplan-Meier test. RESULTS: The overall ten-year EFS was 81.2% and OS 87.6%. Fifty-eight patients from the 330 (17.6%) had metastases at diagnosis, EFS without metastatic process was 84.6% compared to 65.4% with metastasis presented at diagnosis (p = 0.0003), OS was 70.7% compared to 91.2% (p < 0.0001). One hundred and seventy patients (51.5%) were treated with preoperative chemotherapy and/or radiotherapy, whereas 158 patients (47.5%) underwent primary nephrectomy; EFS and OS did not differ: neoadjuvant vs primary nephrectomy EFS was 81.2% vs 80.9% (p = 0.85), OS 89.4% vs 85.4% (p = 0.38). Sixty (18%) patients experienced disease recurrence; OS after relapse was 33%. In the historical group, EFS and OS were 85.7% and 91.2%. In patients treated according to the SIOP 9 protocol, EFS and OS were 68.1% and 74.5%, resp. In patients treated according to SIOP 93-01, it was 83.6% and 93.7%, resp. and in patients treated according to 87 SIOP 2001, it was 7% and 95.4% (p = 0.001 and p = 0.0008), resp. CONCLUSION: WT is a well treatable disease. The aim for the future is to maintain the current very good survival while minimizing the treatment intensity.

Defect-diaphragmatic ratio: a new parameter for assessment of defect size in neonates with congenital diaphragmatic hernia.

AIM: The aim of our study is to introduce a new objective method of perioperative evaluation of the size of diaphragmatic defect to enable comparison of results among various centres and methods used for diaphragmatic reconstruction. MATERIALS AND METHODS: Prospective observational study of neonates with congenital diaphragmatic hernia (CDH) and respiratory distress within 24 h of birth operated on from January 2009 to December 2011. Weight, length, thoracic shape and the diameters of diaphragmatic defect were measured. To determine the relative size of the defect, a defect-diaphragmatic ratio (DDR = defect area:diaphragm area × 100) was calculated. The measured and calculated data were subsequently compared between Gore-Tex patch group (GT) and primary repair group (PR). Mann-Whitney U test was used for statistical analysis. RESULTS: Forty-seven patients with CDH were admitted during study period. The overall survival rate was 79 % (37/47). Preoperative stabilization was achieved in 85 % (40/47). Survival of operated neonates was 93 % (37/40). Diaphragmatic reconstruction with Gore-Tex patch was used in 7 neonates (17 %), and primary repair in 33 (83 %). Mortality in Gore-Tex group was 29 %; mortality in primary repair group was 3 %. Data of anthropometric measurement were complete in 34 children (5 GT and 29 PR). Significant differences were found between GT group and PR group in the size of diaphragmatic defect with the transverse and sagittal diameters of defect (48.0 ± 5.7 vs. 30.1 ± 5.9, P < 0.00061; 34.0 ± 12.5 vs. 16.0 ± 7.3, P < 0.0022) and DDR (18.29 ± 4.60 vs. 5.77 ± 3.28, P < 0.0005), respectively. CONCLUSION: The value of DDR as an objective criterion of the extent of diaphragmatic defect was confirmed by the close correlation between DDR and feasibility of primary repair in the study group. This objective assessment of defect size may improve comparing various surgical techniques and results of different centres, and thus facilitates sharing experience with management of neonates with CDH.

Large diaphragmatic defect: are skeletal deformities preventable?

PURPOSE: The aim of study was to compare growth, nutritional status and incidence of chest wall deformities and scoliosis in survivors of large congenital diaphragmatic hernia (CDH) defect (Gore-Tex patch reconstruction) with survivors with smaller defects and primary reconstruction. MATERIALS AND METHODS: An anthropometric study of 53 children who underwent CDH repair in neonatal period was carried out. Weight, height, and skin-fold thickness were measured, scoliosis and chest wall deformity were evaluated. Body mass index (BMI) and thoracic index (TI) were calculated using standard rules. The measured data were compared with national population standard with the use of standard deviation score (SDS). According to the type of diaphragmatic reconstruction, the patients were divided into two groups [Gore-Tex patch (10) versus primary repair (43)]. Student t test and Fisher exact tests were used for statistical analysis. RESULTS: Pectus excavatum was found in 25 (47%) patients, poor posture in 33% and significant scoliosis in 5%. Compared with the population norm, CDH children had a significantly lower body height SDS (mean -0.39, p < 0.05), weight SDS (mean -0.75, p < 0.001), BMI (mean SDS -0.68, p < 0.001) and lower TI (mean SDS -0.62, p < 0.01). Gore-Tex versus primary repair group significantly differed in incidence of pectus excavatum and BMI (PE: p = 0.027, BMI SDS: p = 0.016). A majority of anthropometric parameters (weight, height, thoracic index, and thorax circumference) and incidence of scoliosis and poor posture in children after Gore-Tex patch reconstruction did not significantly differ from children after primary repair. CONCLUSION: The differences in some anthropometric parameters (weight, BMI, and TI) and in the skeletal deformity suggest that the CDH not only disturbs normal lung growth, but also seems to have implications on some other aspects of somatic development. Whether these changes could be related to the type of diaphragmatic reconstruction or rather to the size of the defect remains uncertain.

[Treatment results in patients treated from 1980 to 2004 for Wilms' tumour in a single centre]. / Lécebné výsledky pacientu lécených v letech 1980-2004 na jediném pracovisti pro nefroblastom.

BACKGROUNDS: The principle behind the treatment of nephroblastoma has been similar for at least 4 decades, based on vincristine and dactinomycine, radiotherapy in selected stages. The last three decades have been characterised by the aim to reduce the intensity and length of treatment. DESIGN: To retrospectively compare survival rates and treatment success in a cohort of patients aged under 19 years, treated from 1980 to 2004 at a single centre by five consecutive treatment protocols. MATERIALS AND METHODS: The outcome was evaluated in patients treated consecutively by two protocols established at the centre before 1980 and modified in 1986, and from 1988 consecutively by three accepted protocols, SIOP9, SIOP93 and SIOP2001. RESULTS: Overall survival as well as event-free survival rates were evaluated by Kaplan-Meier functions in 315 patients (52.7% women). The average age at diagnosis was 3.9 +/- 2.9 years, median 3.3, range 0.01-17.2 years. Age over 12 years in 2.2% patients. The average follow-up time was 13.1 +/- 7.8, median 13.6, range 0.2-27.8 years. The original 104 weeks of protocol KDO86 treatment had a 10-year overall survival rate of 91.9 +/- 3.2%. Overall survival significantly fell with radiotherapy reduction in lower clinical stages and treatment diversification in protocols with substantial treatment length reduction. Overall survival returned to the original value of KDO86 only in 1994, when SIOP93 was accepted with a 10-year overall survival rate of 92.47 +/- 3.0% and event-free survival 85%, with similar trends in the latest protocol, SIOP2001. In the entire cohort two coincident malignancies (tumour duplicities) were found: one B-lymphoma, one neuroblastoma. A second malignancy occurred in one patient--superficial spreading melanoma. CONCLUSION: from the retrospective view the accepted SIOP9 protocol has a significantly worse outcome in both the overall survival and in event-free survival rate compared with the original therapy. Only the SIOP93 and SIOP2001 protocols accepted after 2003 have an acceptable 10-year overall survival rate (around 92%) as well as event-free survival (85%) with substantially reduced length and intensity of treatment, lowering the risk of late effects.

Diagnostic efficacy of ERCP in cholestatic infants and neonates--a retrospective study on a large series.

BACKGROUND AND STUDY AIMS: Cholestatic jaundice in infants is a serious condition, requiring timely and accurate diagnostic evaluation. Our aim was to determine the safety and diagnostic efficacy of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of cholestatic liver disease in neonates and infants. PATIENTS AND METHODS: ERCP procedures in cholestatic infants performed in our endoscopy unit between December 1998 and March 2008 were reviewed retrospectively (n = 104 children, 48 boys, 56 girls; mean age 7 weeks, range 3 - 25 weeks; mean weight 4.05 kg, range 1.5 - 4.8 kg). Endoscopic findings were compared with final diagnoses. Statistical analysis was performed and sensitivity, specificity, positive (PPV) and negative (NPV) predictive values of ERCP were calculated both separately for each diagnosis and on aggregate. RESULTS: Cannulation of the papilla was successful in 95 of 104 patients (success rate 91.3 %). Biliary atresia of any type was found in 51 children (53.7 %), with a sensitivity of 86 %, a specificity of 94 %, a PPV of 96 %, and a NPV of 100 %. Choledochal cysts were found in seven children (7.4 %), with a sensitivity of 100 %, a specificity of 90 %, PPV of 86 %, and NPV of 100 %. Biliary stones were found in seven patients (7.4 %). Other structural pathology was found in six patients, and no abnormality was seen in 24 patients. No severe complications occurred during or after ERCP. CONCLUSIONS: ERCP in cholestatic infants, when performed in an expert center, is a safe and reliable procedure that can detect biliary tract abnormalities (e. g. biliary atresia, bile duct stones or choledochal cysts) with high sensitivity and specificity. Laparotomies can be prevented in infants by demonstrating normal patency of the biliary tract by ERCP or by magnetic resonance cholangiography if improvements in this technique are made.

Dendritic cell-based immunotherapy induces transient clinical response in advanced rat fibrosarcoma - comparison with preventive anti-tumour vaccination.

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.

[Clinical relevance of chromosomal aberrations in bone and soft tissue tumors in children and young adults]. / Klinický význam chromozomálních zmen u nádoru kostí a mekkých tkání detí a mladistvých.

BACKGROUND: We present the results of a cytogenetic and molecular cytogenetic analysis of a series of patients with bone and soft tissue tumors. PATIENTS ANDMETHODS: We analyzed a cohort of 26 patients with Ewing sarcoma/PNET, 15 patients with rhabdomyosarcoma, 5 with synovial sarcoma and one patient with an undifferentiated sarcoma using the cytogenetic and molecular cytogenetic techniques M-FISH and arrayCGH. RESULTS: We found nonrandom chromosomal structural and numerical changes with diagnostic and prognostic relevance in most patients. Eight patients with ES/PNET had only a t(11;22)(q24;q12), eight patients had secondary aberrations as well and six had only secondary aberrations. In the RMS patients we detected the t(1;13)(p36;q14) once and the t(2;13)(q35;q14) four times, both of them characteristic for the alveolar subtype with poor prognosis and numerical aberrations, characteristic for the embryonal subtype, in five patients. Four patients with synovial sarcoma had the diagnostic t(X;18)(p11.2;q11.2), one of them had a complex karyotype with a complex t(X;18;21) (p11.2;q11.2;q11.2) together with t(2;5)(q24-32;p13-14) and t(12;20)(p11;q13). We correlated the karyotype of cancer cells with histopathologic morphologic analysis, clinical outcome and foreign published results. CONCLUSION: Cytogenetic and molecular cytogenetic analysis is a valuable diagnostic tool in bone and soft tissue tumors, especially in less differentiated subtypes, and as such it should be an integral part of curative care.

Surgical management of major pancreatic injury in children.

INTRODUCTION: Major or complicated pancreatic trauma in children is uncommon and management strategies remain controversial. The aim of this study was to evaluate our experience with both early and delayed surgery in these pediatric cases. METHODS: We carried out a retrospective analysis of data of pediatric patients with major or complicated pancreatic injury operated on between January 1994 and December 2005 in our pediatric trauma center. RESULTS: Thirteen children (9 boys and 4 girls) with a mean age of 8.5 years (range 3 - 16 years) were operated for major or complicated pancreatic injury. The extent of injury was: grade II (major contusion without duct injury or tissue loss) in 4 children; grade III (distal transection) in 5 children and grade IV injury (proximal transection) in four patients. Pseudocyst developed in 8 children: 4 with grade II injury, 2 with grade III injury and 2 with grade IV injury (one with abdominal pseudocyst and one with an abdominal and a mediastinal pseudocyst). Early diagnosis and operation was achieved in 5 cases, while delayed diagnosis and operation occurred in 8. Three children underwent cystogastrostomy; 6 had a spleen-sparing distal pancreatectomy and 4 had resection with Roux-en-Y jejunostomy drainage. Endoscopic retrograde cholangiopancreaticography (ERCP) was the most useful diagnostic tool in assessing ductal injury. There were no deaths or long-term morbidity in our group of patients. CONCLUSIONS: Our results support the view that early operation is important in ductal pancreatic injury. We recommend transferring children with a suspected ductal injury to a tertiary center with experience in both pediatric ERCP and pancreatic surgery.

T-tube ileostomy for intestinal perforation in extremely low birth weight neonates.

To evaluate the results of use of T-tube ileostomy in selected cases of intestinal perforation in extremely low birth weight (ELBW) neonates. The records of 288 ELBW neonates treated at author's institution, from 1998 to 2003 were retrospectively reviewed to identify neonates operated for intestinal perforation with T-tube placement. T-tube was inserted into the bowel through the site of perforation or proximally to the perforated gut via separate stab incision. T-tubes were used in five ELBW neonates (BW 600-900 g, gestational age 25-27 weeks) with intestinal perforation, in four of them at the time of primary surgery and in one neonate 8 days after primary anastomosis. All patients survived and there were no serious complications related to the T-tube insertion. Median duration of T-tube placement was 4 weeks (range 3-8 weeks), full enteral feeding after T-tube insertion was achieved in 4 weeks (range 1-6 weeks). All sites of T-tube insertion closed spontaneously. T-tube ileostomy is an effective and safe technique for treatment of selected cases of intestinal perforation in ELBW neonates. With respect to the hypoperistalsis of immature bowel, we recommend the use of T-tube in all cases of isolated intestinal perforation in ELWB neonates.

Congenital diaphragmatic hernia: onset of respiratory distress and size of the defect: analysis of the outcome in 104 neonates.

The purpose of this study was to evaluate the outcome in neonates with congenital diaphragmatic hernia (CDH) either presenting within the first 24 h of life or diagnosed prenatally. The study was particularly focused on the time of onset of respiratory distress and on the use of the Gore-Tex (GT) patch for diaphragmatic reconstruction. Records of 104 neonates with CDH were retrospectively reviewed. The data were analyzed by ANOVA, Kruskal-Wallis test or chi (2) test as appropriate. The result showed that the overall survival rate was 73.1% (76/104). Survival of operated neonates was 91.6% (76/83). Postnatally diagnosed neonates with the onset of respiratory distress within the first minute of life survived in 67%, with the onset between 2 and 10 min survived in 89%, whilst neonates with the onset of respiratory distress after l0 min survived in 100% (P = 0.007). Birth weight, gestational age, time of onset of respiratory distress and Apgar score significantly differed between survivors and nonsurvivors. Primary closure of the diaphragmatic defect was performed in 62 patients while the GT patch was used in 21 patients. The survival of patients with a large defect treated with a GT patch was lower (76.2 vs. 96.8%, P = 0.003). There was only one case of recurrence in our series with the GT patch. Survival depends on the time of onset of respiratory distress and size of the defect, both of which correlate with the degree of pulmonary hypoplasia. The term high-risk CDH is appropriate only for children with respiratory distress within the first 10 min of life and those diagnosed prenatally. The GT patch is a suitable material for the diaphragmatic reconstruction; we suppose that the recurrence is caused by incorrect attachment of the patch to the thoracic wall.

Acute gastro-intestinal obstruction as a late presentation of congenital diaphragmatic hernia. A report of three cases.

Three cases of acute gastro-intestinal obstruction due to incarceration of congenital diaphragmatic hernia (Bochdalek hernia) in infants are reported. The level of incarceration was stomach, small and large intestine. All of the presented posterolateral diaphragmatic defects were small and without a sac. History of trauma was absent in all patients. Two girls recovered well while a boy died of intracerebral bleeding one month after surgery. Acute gastro-intestinal obstruction as a late, post neonatal, presentation of congenital diaphragmatic hernia is a rare, life-threatening emergency. The combination of gastro-intestinal obstruction, circulatory and respiratory distress requires urgent gastro-intestinal decompression, fluid resuscitation and ventilatory support. Surgery can be performed safely only after pre-operative stabilization.

Porcelain gallbladder with extrahepatic bile duct obstruction in a child.

An extrahepatic bile duct obstruction was diagnosed in a 13-year-old boy presenting with pruritus, abdominal pain and jaundice. Several weeks after sphincterotomy and biliary stenting via endoscopic retrograde cholangiopancreaticography which relieved the obstruction, the patient was operated on. Severe fibrosis encased the extrahepatic biliary tract, so only cholecystectomy was performed because planned hepaticojejunoanastomosis could jeopardize the vascular supply to the liver. Histopathology showed calcification of the gallbladder wall and chronic fibroproliferative changes in the surrounding tissue. The stricture of extrahepatic biliary duct resolved after 3 years of repeated replacement of stents. The stenting was thereafter terminated. In the following 3 years no dilation of intrahepatic bile ducts and no laboratory signs of cholestasis recurred and the now 19-year-old boy is doing well. Neither a case of porcelain gallbladder with extrahepatic bile duct obstruction in a child nor a successful treatment of the obstruction by long-term stenting has been described in the literature yet.

Congenital diaphragmatic hernia associated with esophageal atresia, tracheoesophageal fistula, and truncus arteriosus in a premature newborn.

The occurrence of coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is extremely rare and is considered highly lethal. The combination of CDH with EA/TEF and truncus arteriosus communis (TAC) has not been reported in the literature to date. The authors describe a premature neonate with this association.

[Current results in treatment of omphalocele and gastroschisis]. / Soucasné výsledky lécby omfalokély a gastroschízy.

OBJECTIVE: The aim of the study was to evaluate current possibilities of treatment of newborns with abdominal wall defects: omphalocele and gastroschisis. SETTING: Department of Pediatric Surgery, 2nd Medical Faculty Charles University and Faculty Hospital Motol, Prague. METHODS: A retrospective cohort study of the treatment results in newborns with defect of abdominal wall. RESULTS: Thirty eight newborns with omphalocele and gastroschisis were treated at the authors department in the period of 1995-2002. Primary closure of defect was possible in 23 newborns (60%), Goretex patch or "silo" technique was used in 13 patients (34%), while two newborns (6%) were not operated on. The overall survival in children with omphalocele was 73%, being 89% in children without chromosomal anomalies. Six infants died: three with trisomy 13, one with trisomy 18, one infant died of sepsis and one had primary pulmonary hypertension and congenital heart defect. The survival in children with gastroschisis was 94%, one child died of multiorgan failure during sepsis. CONCLUSION: Nowadays prenatal diagnosis of gastroschisis or omphalocele is a recommendation for complex examination and consultation in a specialized center. The prognosis for most isolated defects of abdominal wall is good as far as survival and quality of life are concerned.

[Are there any new procedures for treating necrotising enterocolitis in neonates with very low birth weight?]. / Existují nové postupy v lécbé nekrotizující enterokolitidy u novorozencu velmi nízké porodní hmotnosti?

OBJECTIVE: To evaluate some perinatal and neonatal risk factors of occurrence of necrotising enterocolitis (NEC) and the current possibilities of treatment. DESIGN: A retrospective data analysis. SETTING: Mother and Child Care Institute, Prague. METHODS: The clinical course in 28 neonates treated for NEC on the worksite of the authors in the period 1999-2003. Only neonates with NEC in the second and third Bell's stages were included in the group. RESULTS: From a group of 14,275 neonates born during the reference period, NEC was diagnosed in 28 neonates. In all cases, they were neonates with very low birth weight (VLBW). The average gestation age was 26.6 weeks (the range was 23-35; the median was 25.5); the average birth weight was 822 grams (the range was 340-1,490g; the median was 735). Incidence was between 1.08 and 2.37/1,000 live births (the average was 1.94). On the average, NEC occurred on the 17th day after birth (the range was 5-59; the median was 14). 14 neonates (50%) in the acute stage of NEC were operated on; 9 children (32%) were only treated conservatively. In eight children at the acute stage, who were operated on, divided ileostomy was created; in four children, a T-drain was used for removal of the intestinal content and an abdominal drain was used for this purpose in one child. Repeated surgery was necessary in five children. Of the conservatively treated ones, five children recovered as a result of conservative treatment and four children died as a result of fast progression of NEC without surgical treatment. All the children treated for patent ductus arteriosus (PDA) with Indometacin died. The overall mortality in the group was 39.3% (11 children died). CONCLUSION: Prematurity seems to be the common denominator of the occurrence of NEC. Treatment of NEC in highly immature neonates with birth weight below 1,500g requires comprehensive cooperation of a neonatologist and a children's surgeon. Resection of a necrotic intestine with creation of a stomy remains a standard procedure, which is followed in unstable neonates with a localised form of the illness. Prevention of premature birth, antenatal steroids, frequent feeding (trophic feeding) and the corresponding correction of water management seem to be a reasonable strategy to reduce the incidence of NEC. The resulting incidence, mortality, the time of the occurrence of NEC and the therapeutic procedures are within the limits stated in the international literature.

[Necrotizing enterocolitis in extremely immature newborns. The surgical treatment potential]. / Nekrotizující enterokolitida u extrémne nezralých novorozencu--moznosti chirurgické lécby.

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.

[Treatment of the Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine, from 1979 to 2004]. / Lécba Hirschsprungovy choroby na Klinice detské chirurgie 2. LF UK a FN Motol v letech 1979-2004.

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.

[Surgery of congenital defects--present trends]. / Chirurgie vrozených vad--soucasné trendy.

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.

[Reconstruction of the diaphragm with a polytetrafluoroethylene patch in neonates with congenital diaphragmatic hernia]. / Rekonstrukce bránice polytetrafluoroetylenovou záplatou u novorozencu s vrozenou bránicní kýlou.

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.

[Treatment of hypospadias in childhood]. / Lécba hypospadie v detském veku.

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.