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Objectives: Respiratory morbidity in congenital diaphragmatic hernia (CDH) is associated with high mortality and adverse outcome. Accurate prenatal diagnosis is essential for prognosis and potential treatment in utero. The aim was to evaluate the prenatal ultrasound findings in assessing the respiratory prognosis in fetuses with isolated left-sided CDH., Methods: We retrospectively analyzed the medical records of 59 prenatally diagnosed left-sided CDH cases managed at a tertiary perinatal center., Results: Survival rate in the study group was 73% (43/59). We found no statistically significant relationship between survival and the presence of polyhydramnios, gestational age at diagnosis, lung-to-head ratio (LHR) and observed/expected LHR (O/E LHR) values, gestational age at birth and birth weight. Intrathoracic liver herniation was a statistically significant parameter adversely affecting survival (37.2% in survivors, 68.8% in non-survivors, p = 0.031) and logistic regression confirmed this relationship. The presence of pneumothorax and severe pulmonary hypertension were significantly associated with mortality (82% non-survivors versus 15% in survivors, p = 0.0001)., Conclusion: Intrathoracic liver herniation seems to be a reliable parameter in the prediction of survival and neonatal respiratory morbidity in fetuses with isolated left-sided CDH. In contrast, we found no significant correlation between perinatal outcome and LHR, O/E LHR values, birth weight and gestational age.

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.

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.

Hepatoblastoma is the most common primary hepatic tumor in children, and only a limited number of detailed karyotypic analyses have been reported to date. In the present study, cytogenetic abnormalities were identified in nine cases of hepatoblastoma from a single institution. Among characteristic chromosomal changes detected were simple numerical aberrations, structural alterations of chromosomes 1, 2, and 8, and the recurrent unbalanced rearrangements der(4)t(1;4)(q25.2;q35.1) and der(6)t(1;6)(q21;q26). Array comparative genomic hybridization was applied in four of the cases. The combined cytogenetic, molecular cytogenetic, and histopathologic analyses are presented here, together with clinical data. The results substantially confirm previous findings of aberrations involving chromosomal loci on 1q, 2 or 2q, 4q, 6q, 8 or 8q, and 20 as significant in the development and clinical course of this disease.

Objective: The aim of this article is to review the current options of prenatal diagnosis in congential diaphragmatic hernia (CDH)., Subject: Systematic review., Setting: Institute for the Care of Mother and Child, 3rd Medical Faculty, Prague., Subject and Method: Review of recent published data., Conclusion: The basic method for prenatal diagnosis of congenital diaphragmatic hernia is 2D ultrasonography: measurement of lung-to-head ratio (LHR), observed to expected lung to head ratio (observed to expected LHR - O/E LHR), localization of diaphragmatic defect, assessment of liver position and presence of associated anomalies (negative prognostic factors). Prenatal diagnosis can be supplied with 3D ultrasonography and magnetic resonace (imaging methods for valid measurement of fetal lung volume and/or presence of associated congenital defects confirmation). The reactivity of intrapulmonary arteries are evaluated by hyperoxygenation test and measurement of arterial Doppler parameters. Isolated diaphragmatic hernia is not an indication for invasive prenatal diagnostic methods. Important part of succesful prenatal diagnosis and therapy of CDH is concentration of cases in specialized centre.

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.

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.

Aim: Assessment of surgical complications and options for their therapy following replacement of oesophagus by stomach. METHODOLOGY AND MATERIAL: A retrospective study of clinical and pathological data collected from 25 children, who underwent replacement procedures of their oesophagus by stomach during the 1992-2005 period. 18 subjects were indicated for the procedure for oesophageal atresia with large stubs distance and 7 subjects were indicated for extensive oesophageal strictures following acid burns., Results: The study group included 25 children whose mean age at the time of the procedure was 12.5 years (the age range 8 to 13.3 years). Early postoperatively, a fistule in the oesophageal anastomosis region was confirmed in 7 subjects (28%), however, in all cases it healed successfully on conservative management. In five cases (20%), a stricture in the pyloric region developed and in 4 cases it was managed using balloon dilation. During the long-term follow up period, oesophageal anastomosis strictures were diagnosed in 9 children (36%). The problem was managed using repetitive balloon dilations. One child was operated for ileus with adhesions and in one child, a nutritional jejunostomy had to be re-conducted. One girl exited at home, a year following the procedure, due to respiratory arrest on aspiration., Conclusions: Transposition of the stomach is a suitable method of choice for oesophageal replacement in childhood, providing good quality of life for majority of patients. Early and late surgical complications correspond with a degree of seriousness of the disorder and require treatment and long term follow up to be conducted in a specialized clinic.

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.

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.

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.

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.

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.

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.

Background: The aim of the study was to analyze morality, incidence of complications and influence of various prognostic factors on the outcome of patients with esophageal atresia. A retrospective analysis of the records of 109 consecutive cases of esophageal atresia and/or tracheoesophageal fistula operated in 1992-2001 was undertaken. Primary anastomosis was achieved in 68 (62.4%) patients, in 7 (6.4%) procedures aimed at esophageal substitution in future were performed and in 34 (31.2%) delayed anastomosis was primarily planned., Methods and Results: Major associated anomalies were found in 55 (50.4%), cardiac anomalies in 19 (17.4%), and more than one associates anomaly in 16 (14.7%) patients. The most frequent surgical complication was anastomotic leak in 11.9%, septic complications were the most frequent and occurred in 66 (60.5%) patients. Gastroesophageal reflux was diagnosed in 30.3% and treated surgically in 14.7% of infants. The overall mortality was 20.2% (22 patients). The most frequent cause of death was sepsis and/or pneumonia. Long gap esophageal atresia was not found to be a significant factor in mortality. Factors significantly increasing mortality were birth weight below 2000g (OR 3.94), cardiac anomalies (OR 3.95), and mechanical ventilation before the operation (OR 2.97)., Conclusions: Analysis of the outcome suggests that improved strategy in treating infants with multiple anomalies and more frequent attempts at primary anastomosis even in children in sub optimal general condition at diagnosis could decrease mortality.

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.

Perforation of the stomach caused by a wedged in foreign body is a rare complication in children. The authors present the case-report of a 15-year-old girl with anorexia nervous who did not disclose that she had swallowed a spoon and after 6 months was operated on account of perforation of the stomach. In the discussion the authors summarize the problem of swallowed foreign bodies in children and the problem of gastric damage in anorexia nervosa.

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.

Objective of Study: Evaluation of success of extracorporeal membrane oxygenation, EMCO) in the treatment of acute respiratory failure in mature neonates., Type of Study: Clinical pilot study of the EMCO centre., Setting: Institute for the Care of Mother and Child, Prague., Material and Method: In 12 consecutive patients with severe acute respiratory distress syndrome and/or circulatory failure of different etiology who met the indication criteria the method of venoarterial EMCO was used., Results: Venoarterial extracorporeal membrane oxygenation was successful in 75% patients who survived. Four patients died (1x syndrome of cerebral death associated with severe hypoxic-ischaemic encephalopathy, 2x severe irreversible haemorrhage, into the abdominal and thoracic cavity, 1x periventricular intraventricular haemorrhage grade III.). The mean period of EMCO was 71.4 +/- 31.7 hours (range 25-130 hours). On comparison of the surviving group (S) and the non-surviving group (NS) there was a significant difference in the necessity of continuous inotropic adrenaline support during EMCO. In patients who died necropsy confirmed irreversible multiorgan failure., Conclusion: In indicated cases extracorporeal membrane oxygenation remains the method of choice in critically ill mature neonates. A prognostically adverse factor is the necessity of inotropic support and haemodialysis during EMCO.

The authors evaluate their experience with the diagnosis and treatment of abdominal lymphangiomas during the period of 1995-1999. During the above period 6 girls and 4 boys with abdominal lymphangiomas were operated. The mean age at the time of operation was 5 years and 8 months. Ultrasonographic examination was made in all 10 patients, CT examination in 8. The surgical finding was lymphangioma of the mesenterium 4x, of the omentum 2x, of the adrenals 2x of the retroperitoneum 1x and intestinal lymphangiomatosis 1x. Macroscopically complete extirpation of the lymphangioma was possible in 9 patients and called for resection of the gut 3x and for adrenalectomy 2x. In one patient subtotal extirpation was performed. Postoperative follow-up did not reveal a relapse in any of the children. Abdominal cystic lymphangiomas are rare benign malformations of the lymphatic system. For preoperative differential diagnosis in typical cases USG is sufficient. The therapeutic method is complete surgical extirpation which is associated with a low incidence of relapses.

Objective of Study: a) assessment of the relationship between ileus of premature infants and the development of intestinal perforation in premature infants, b) suggestions of an optimal therapeutic procedure. In the study children (n = 50) are included a) with intestinal perforation in conjunction with impaired excretion of meconium (n = 22), b) with an ileus state based on obstruction of the ileum by a viscous meconium treated either surgically or conservatively (n = 28). Surgical treatment involved: a) establishment of a double ileostomy (n = 28), b) insertion of a T drain into the terminal ileum (n = 8), c) removal of meconium from the gut and its primary closure (n = 2). Conservative treatment in 11 children involved irrigography with an liquid contrast substance under X-ray control. The group of children with perforation was compared with the group of children without perforation, risk factors were evaluated by statistical methods. The necessity of ventilation (P = 0.051) and gestation age (P = 0.006) proved to be statistically significant risk factors for the development of perforation. Survival was not influenced by perforation. All 11 children treated conservatively survived, of 39 operated children 26 survived (66.7%). An early start of conservative treatment of ileus of premature infants reduces markedly the risk of intestinal perforation and can thus influence the survival of low birth weight neonates.

The objective of the investigation was to evaluate the success of a new therapeutic protocol in patients with congenital diaphragmatic hernia (CDH). During the period from 1/1994 till 12/1998 41 patients with CDH were admitted. In 36 patients (88%) left-sided CDH was diagnosed, in 4 patients (10%) right-sided CDH and one neonate (2%) bilateral CDH. Fifteen cases (37%) of CDH were assessed prenatally. Twenty-two children (54%) were treated by inhalation of nitric oxide (INO) and 4 patients (10%) by extracorporeal membrane oxygenation. The total incidence of associated developmental defects was 20% and the total mortality 34%. On comparison of the surviving (group S, n = 27) and the non-surviving NS, n = 14) patients statistically significant differences were found in the Apgar score during the first minute (S: 5.9 +/- 0.5 vs. NS: 3.4 +/- 0.7, p < 0.008), in the oxygenation index (OI) two hours after admission (S: 11.9 +/- 2.9 vs. NS: 27.7 +/- 8.3, p < 0.03), in the alveolo-arterial oxygen difference (AaDO2) 2 hours and 12 hours after admission (S: 369 +/- 47 torr and 237 +/- 47 torr resp. vs. NS: 552 +/- 29 torr and 557 +/- 26 torr resp., p < 0.02) and in the need to start extracorporeal membrane oxygenation (S: 3.7% vs. NS: 21.4%, p < 0.009). The investigation confirmed a reduced mortality of neonates with CDH by introducing new therapeutic methods. Risk factors are early prenatal diagnosis, the presence of associated developmental defects, high values of oxygenation and ventilation with the necessity to start nitric oxide inhalation.

The authors present an account of their experience with oesophageal replacement by gastric transposition in 10 children during the period between November 1992 and January 1999. Indications for oesophageal replacement was in seven children atresia of the oesophagus at the age of 9-36 months and in three children (2.5, 3 and 14 years) a long stricture of the oesophagus after caustic injury which did not respond to balloon dilatation. Replacement after caustic injury was made in children aged 2.5, 3 and 14 years. In eight patients the replacement was made without thoracotomy, by blunt resection of the mediastinum, in two patients after caustic injury thoracotomy had to be performed. In three patients after surgery a temporary fistula developed at the site of anastomosis which healed spontaneously. Eight patients are in a satisfactory condition after surgery, one patient five months after operation swallows with difficulty, his birth weight was 1500 g. One female patient died one year after oesophageal replacement, apparently due to aspiration and respiratory arrest. Gastric transposition is a considerate and relatively simple method in child age. The functional results are favourable.

The authors give an account of their experience with the treatment of primary lung tumours in children during 1989-1998. Primary lung tumours in children are extremely rare and represent a varied spectrum of pathological conditions (pneumoblastoma, rhabdomyosarcoma, mucoepidermoid carcinoma, pulmonary endodermal tumour and benign tumours). During the mentioned period 10 children were operated on account of primary lung tumours, 5 with malignant tumours and 5 with benign tumours. In these patients during the above period 3 pneumonectomies, 5 lobectomies and 3 segmental resections were made. Chemotherapy was indicated in children with pneumoblastoma and rhabdomyosarcoma. Two patients with pneumoblastoma died, 3 children with malignant and 5 children with benign tumours live without symptoms of the disease.

The diagnosis and treatment of congenital diaphragmatic hernia, CDH, is an interdisciplinary problem. The patient concentration with prenatally diagnosed CDH for comprehensive examination with subsequent decision on the type of delivery improves the prognosis of patients with CDH. Introduction of corticoid therapy in prenatally assessed CDH reduces dysfunction and surfactant deficiency and can reduce the degree of respiratory failure during delivery. In very early prenatally assessed CDH it is possible to consider the possibility of combined corticoid and TRH (thyroxin releasing hormone) treatment of CDH. The authors submit also contemporary possibilities of prenatal intervention treatment of CDH (indication criteria for reconstruction operations of foetal surgery, intrauterine closure of the trachea and artificial laparoschisis). The concentration of patients with postnatally diagnosed CDH and a severe grade of acute respiratory insufficiency in a department with conventional and non-conventional artificial pulmonary ventilation incl. inhalation of NO oe extracorporeal membrane oxygenation, ECMO, is a further step towards optimation of treatment. Continuous evaluation of parameters of pulmonary functions during the pre- and postoperative period may prove that a therapeutic protocol with delayed surgery is useful.