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Purpose: To describe the presence of bladder malformations in a surgically induced model of myelomeningocele (MMC)., Methods: A MMC like defect was created in the mid gestation using the previously described model in sheep. Bladders were examined macroscopically and histopathological changes were assessed using H-E., Results: Non prenatally corrected animals presented dilated bladders and separation between muscle bundles. Those malformations were not found in corrected animals or controls., Conclusions: Some bladder changes can be described in a surgically-induced model of MMC. These changes could be prevented using open fetal surgery.

Aim: To describe central nervous system malformations in the surgically induced model of Myelomeningocele (MMC) and their prevention using different prenatal treatments., Methods: MMC was surgically created in 33 fetal lambs. Fifteen did not undergo fetal repair (group A). Of the lambs that did undergo repair, 10 were repaired with open two layer surgical closure (group B), 5 with fetoscopic coverage using bioglue (group C) and 3 fetoscopically using a patch (group D). All procedures were recorded and lamb brains and spinal cords were examined grossly and microscopically in coronal sections for structural organization anomalies. Histopathological changes were assessed using HE and S-100 neural marker., Results: Hydrocephalus, Arnold-Chiari type II (AC-II) malformation and some neuronal migration disorders were observed in group A. Brains from group B and D were not hydrocephalic and had neither cell migration disorders nor hindbrain herniation. Group C presents mild degrees of hydrocephalus and AC-II. In group C lumbar lesion was covered by fibrous tissue., Conclusions: Some of the central nervous system abnormalities observed in human disease are present in the surgically induced model of MMC. In this model avoidance of fluid drainage using open fetal surgery limits malformation severity.

Aim: To analyze morbility, mortality and neonatal intensive care management in CDH patients who required a prosthetic patch to close the diaphragmatic defects, and to compare these results with those who were made a primary closure., Material and Methods: We reviewed the clinical charts of CDH patients managed at our institution between January 1994 and December 2006, including demographic data, clinical management, treatment options, complications and mortality. Appropriate statistical tests were used to evaluate the data: mortality, need of high frequency oscillatory ventilation (HFOV), days of intubation, days of total parenteral nutrition (TPN), days of admission, reherniation, need of Nissen funduplication and intestinal obstruction; a p value less than 0.05 was considered statistically significant., Results: Eighty-seven CDH patients were reviewed, 68 right, 17 left and 2 bilateral defects. Seventeen died before surgery (19.5%). Among the 70 operated patients, 21 (24.1%) required a prosthetic patch, 18 in the left and 3 in the right side. Overall mortality, need of HFOV, intubation days, need of TPN, days of admission, reherniation rate and need of Nissen funduplication were all significantly worse in the group who required a patch. Rate of intestinal obstruction was similar in both groups., Conclusions: Patients who required a prosthetic patch to close the diaphragmatic defect suffered from higher morbidity and presented a higher mortality than those who did not required the patch.

Objective: To analyse our results on liver transplantation (LTX) in primitive malignant unresectable liver tumours in children and discussing its controversial indications in order to our experience., Methods/patients: We report 12 patients with ages ranging from 6 months to 14 years old. They had hepatoblastoma (11) and fibrolamellar hepatocelullar carcinoma (1) without cirrhosis. LTX was considered as primary treatment in 10 patients (PRETEXT IV or any grade if extension to retrohepatic cava vein, 3 hepatic veins or porta vein were assessed) and as rescue therapy after recurrence (1) or persistence of unresectable macroscopic rests (2). One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We used entire liver (5), left lateral segment from cadaveric donor (3), live related donor (3, 2 segments II-III and 1 right liver) and left lateral segment from split (1). All children received chemotherapy prior and post-transplantation following SIOPEL protocol. OUTCOMES ANALYSED: Procedure tolerance, survival, recurrence rate, disease-free period and risk factors for adverse evolution., Results: All patients overcame the LTX and no early loss of the graft was assessed. 2 patients died because of tumoral relapse, 1 after primary LTX and 1 after rescue LTX (survival rate of both groups 90% vs 50%). Graft and patients 1-year, 3-year, 5-year and 14-year survival were 91%, 91%, 82% and 82% respectively. The boy who presented lung metastases developed new ones one year after LTX that were removed and he actually is free of disease. The disease-free period has a probability for 1, 3 and 5 years of 91%, 75% and 75%. Tumoral tissue persistence is the only risk factor for an adverse evolution in our series., Conclusions: LTX is possible therapeutic approach for unresectable malignant liver tumours. It provides better results as a primary treatment than as a rescue one, being these outcomes comparable to those from resectable tumours. A right staging and referring patients to an expertise centre contribute to optimize results. LTX for patients presenting with lung metastases could be a controversial option. Live-related donor transplantation is an excellent alternative to avoid disease progression during cadaveric waiting list.

Background: Orthotopic liver transplantation (OLT) in children younger than one year is associated to higher waiting list mortality and alternative graft sources are required. We present our experience with this particular group of age., Methods: Infants younger than one year who received an OLT between 1986 and 2005 were reviewed focused on graft and children survival depending on period and type of graft. Periods were 1:1986-1995; 2:1996-2000 and 3:2001-2005. We also evaluate cold ischemia time (CIT), graft lost causes and differences between CIT and anhepatic time (AT) depending on graft type., Results: Eighty-three children received 103 OLT. Liver transplant indications were 59 (72%) biliary atresia, 8 (10%) metabolic causes, 6 (8%) liver failure, 3 (4%) cirrhosis and 7 (6%) miscelaneous. Patient and graft survival after 5 years was increased depending on period: 45% and 65% on period 1, 70% and 80% on period 2, 94% y 97% on period 3 (p < 0.0198). Thirty-seven grafts were reduced lobes (42%); 8 (21%), 17 (45%) and 12 (35%) during periods 1, 2 and 3 respectively and their 5 years survival rate was 68%. Twenty-four were whole grafts (31%); 11 (45%), 10 (45%) and 3 (14%) during periods 1, 2 and 3 and their 5 years survival rate was 63%. Fourteen grafts were living-related donor (16%); 1 (7%), 2 (14%) and 11 (79%) during periods 1, 2 and 3 and their 5 years survival rate was 93%. Eight (11%) were split; 0, 1 (12%) and 7 (90%) during periods 1, 2 and 3 and their 5 years survival rate was 100%. Average CIT depending on graft was: living donor 5,5 hours (IQR: 4-7), split 6,1 hours (IQR: 5-8), whole 9.2 hours (IQR: 6-11) and reduced 8.5 hours (IQR: 6-11) (p < 0.05). Average AT depending on graft was: living donor 1 hour (IQR: 0.5-1.5), split 1 hour (IQR: 0.5-1.4), whole 1,1 hours (IQR: 0.5-1.5) (p > 0.1). Twenty-four grafts were lost (28%): 10 (41%) were surgical related causes and 6/10 (60%) of them were whole grafts., Conclusions: Survival rates in children younger than one year are similar to another groups of age. There was a significant increase on graft survival according to transplantation group experience. A higher rate of graft lost is associated to whole grafts. Most frequent reasons of graft lose were related to sepsis and immunosuppresion. A significant shortening of CIT is observed in related living donor and split grafts.

Purpose: (PD), possibility of regresion and hidden mortality are open questions in congenital cystic adenomatoid malformation (CCAM) treatment., Methods: Children with CCAM were reviewed focused on: PD, postnatal diagnosis, clinic, radiology, histology and evolution., Results: Seventeen fetus had PD of CCAM. Five gestations were electively finished (41%) with PD of CCAM previous to 20th week, 3/5 (60%) were type III and 2/5 (40%) type I; 4/5 (80%) presented mediastinal shift and 1/5 (20%) hidrops. Two fetuses (11%) suffered fetal demise in 20th and 32th week; 1 type 1 and 1 type III; 1/2 (50%) presented hidrops and 2/2 (100%) mediastinal shift. Two (12%) died before 24 hours after birth without intervention possibility due to respiratory instability, 1 type II and 1 type III, both with mediastinal shift (100%). In one fetus with a type III malformation the image disappeared completely in 32th week and no intervention was done. Fourteen patients were operated (8 girls and 6 boys); 7/14 (50%) had PD, average diagnosis week was 21.9 (range 19.1-35.5), 5/7 (71%) was type I, 1/7 (14%) type II and 1/7 (14%) type III. None had mediastinal shift or hidrops. Average postnatal diagnosis week was 7 months (range 0.1-29). In 10/14 (71.4%) there were not respiratory difficulty during neonatal period and 3/10 (30%) suffered respiratory infections afterwards. Average week of operation was 8 months (range 0.1-30). PD was according with histology in 6/7 (86%) patients. After an average follow-up period of 4.3 years (range 1-9.5) the only complication is a pectus excavatum., Conclusions: More than half of patients with PD of CCAM died without intervention. Half of cases of CCAM are diagnosed prenatally. Type of CCAM in PD is according to histology in 86% of the cases. Fetuses with hidrops present a worse prognosis. Surgical timing do not seem to influence on outcome.

Background: The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years., Material and Methods: Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated. Nineteen RMS were excluded because they were treated by oher departments. The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests., Results: 1. RMS: Median age at diagnosis was 2.3 years (range 6 m-16y). Twelve were genitourinary, 3 thoracic, 3 abdominal, 1 was located in limb and 1 in the neck. Histologically, 13 were embryonal, 5 botryoid, 1 alveolar and 1 fusiform. At diagnosis, 74% were in stages I or II. Fine needle aspiration biopsy (FNAB) was made in 5 children and the result was always imprecise or mistaken. Surgical biopsy was made before the definitive surgery in 12 cases. In the remaining 8 children the diagnosis was made only after surgical resection. With an mean follow-up of 70 +/- 43 moths, 6 children died. The prognostic factors associated with poor outcomes were genitourinary location, non radical excission, the presence of distant metastases at onset and alveolar histology. 2. Other sarcomas: Median age at diagnosis was 10.9 years (range 4 days-15 years). Among this group, there were 6 fibrosarcomas, 4 indifferentiated sarcomas, 3 synovial sarcomas, 2 abdominal desmoplastic small round cell tumours, 2 neurofibrosarcomas and 1 leiomyosarcoma. Only 9 received chemotherapy and one radiotherapy. All but one were operated. Five out ot the 19 died., Conclusions: Although the role of surgery is crucial, it is necessary to refine the initial histological diagnosis, because neither the PAAF or the biopsy have always been correct. The negative prognostic factors in our series were metastases present at diagnosis, genitourinary location and alveolar (RMS), desmoplastic or indifferenciated histology.

Background: Congenital portosystemic shunt (CEPS) is a rare condition that was first reported by John Abernethy in 1793. Two types of CEPS are described: type I (side to end anastomosis) or congenital absence of the portal vein, and type II (side to side anastomosis) with portal vein supply partially conserved. Type I CEPS is usually seen in girls and associates multiple malformations as polysplenia, malrotation, and cardiac anomalies. Type II is even rarer with no sex preference and no malformations associated. Hepatic encephalopathy is a common complication of both types in adulthood. Liver transplantation is the only effective treatment for symptomatic type I CEPS. A therapeutic approach for type II could be surgical closure of the shunt., Objective: To analyse our experience in diagnosis and management of portosystemic shunts., Methods: We report 4 cases of CEPS (3 type I and 1 type II) diagnosed between January-1997 and March-2005 in our department., Results: We present 4 patients with ages at diagnosis ranging from 0 to 28 months, 3 type I CEPS (2 boys and 1 girl) and 1 boy type II. The type I girl was prenatally diagnosed at 12 weeks of gestation. Initial clinical signs in type 1 boys were splenomegaly and hypersplenism, both with normal pondo-statural growth. No polysplenia or cardiac anomalies were assessed. One of them presented mild developmental delay, dismorphic features and facial telangiectasias. He had normal coagulation tests with chronic hepatic dysfunction (high transaminases) and regenerative nodular lesions were seen by imaging techniques. The other type I patient had hypoprothrombinemia, tendency to capillary bleeding (haematomas and epistaxis) with preserved liver function. Both patients have developed mild portal hypertension and present steatosis signs at liver biopsy. The type I girl presents a 21 trisomy and associates a cardiac anomaly (interauricular communication). Her hepatic function test are normal but liver calcifications can be seen by ultrasound. Type II child associates hypospadias but he has no clinical sigh or symptom related to the shunt. In our three cases diagnosis was suggested by conventional and Doppler ultrasound and confirmed by angio-resonance imaging. All our patients are included in a meticulous clinical and radiological follow-up with no need of surgical treatment for the shunt until now., Conclusions: Although diagnosis of these malformations could be casual we have to think about CEPS in children presenting unspecific liver disease. Magnetic angio-resonance imaging is actually the best diagnosis methods for CEPS. These patients have a high risk for developing hepatic encephalopathy and portal hypertension, so a careful follow-up is required although surgery is not usually needed until adulthood.

Unlabelled: Esophageal replacement is a surgical procedure rarely indicated in children. It is used in esophageal atresia type I and long-gap atresia when anastomosis is not possible, corrosive strictures and other unusual causes. Type and location of the graft depend on etiology and surgeon preferences. We analyse our results of a large series of esophageal replacement., Methods: . We reviewed esophageal replacements carried out in our department between January-1992 and December-2004. We report 29 patients (15 girls and 14 boys) with ages ranging from 2 months until 14 years old (median 24 months). 11 (37.9%) had esophageal atresia type I, 7 (24.1%) long-gap esophageal atresia, 8 (27.5%) caustic esophagitis, 1 herpetic esophagitis, 1 candida esophagitis and 1 esophageal necrosis due to sclerotherapy. Colon was used for substitution in 25 cases (86.2%) and stomach in 4 (13.8%). Graft location was retromediastinal in 25 children (86.2%), retrosternal in 3 and subcutaneous in 1. Native esophagus was removed in all but 2 out of 3 retrosternal cases., Results: After a follow up between 7 and 145 months (median 76 months) all children have a functional graft. Actually all patients tolerate oral feeding in a satisfactory way, and have had a normal pondostatural growth. Post-operative complications were pyloric obstruction in 3 patients (10.3%), upper anastomosis stricture in 3 (10.3%), 2 (6.9%) surgical wound evisceration, 2 (6.9%) diaphragmatic hernia, 1 (3.4%) retro-mediastinal abscess and 1 (3.4%) colo-gastric emptying difficulties. Also 2 pleural effusions, 1 cervical wound abscess and 1 abdominal wound one. Re-operation was needed in 11 patients (38%) due to these adverse events. Other complications were conservatively solved: 6 (20.7%) salivary fistula, 1 intestinal suboclusion and a dumping syndrome. One girl died due to a mycotic mediastinal abscess with perforation of the aorta 11 days after surgery. Overall survival was 96.5%., Conclusions: Esophageal replacement has limited indications. It allow a good functional result, with adequate oral feeding and normal growth. We believe that both colon and stomach have similar outcomes, but gastric pull-up is easier to perform. It is a major surgery whose risk of complications is higher in early post-operative time.

Aim: In this study, we analyze our results in the treatment of congenital diaphragmatic hernia (CDH) using conventional ventilatory therapy without ECMO., Patients and Methods: fifty one CDH patients (27 males and 24 females) treated at our institution between 1997 and 2004 were reviewed. Data referred to obstetrics, prenatal diagnosis, newborn care, type of hernia and surgical treatment were analyzed. We recorded ventilatory treatment modalities and the outcome of the patients. We also compared the survival of our series with those expected using the formula proposed by the CDH study group in 2001. We finally analized separately those patients with early clinical presentation and who fulfilled ECMO criteria. Data from necropsies were also recovered when available., Results: Prenatal diagnosis was made in 58% of the patients. Fifty nine percent were born by c-section. The diaphragmatic defect was left-sided in 42 patients, right in 8 and bilateral in 1. Ten patients needed a prosthetic patch to close the defect. Eighteen out of the 51 patients (35%) died, 11 of them without surgical treatment. Early presentation of clinical picture was evident in 44 patients; among them 46% required high frequency ventilation and 53% nitric oxide therapy. Medium age at operation was 56+/-49 hours. The 7 children with late clinical presentation are alive. Among the 44 remaining patients, 26 are also alive (59,09%), data similar to those expected by the formula (62.39%, p>0.05). Fifteen patients had oxigenation index (IO) over 40, with a stimated survival rate of 48%, a statistically significant lower rate when compared to patients with IO<40. None of the patients with IO>40 survived; in 4 out of the 7 available necropsy studies, a severe lung hypoplasia was found (index lung weight/body weight <0.006). Among the remaining 27 children with IO<40, 24 (90%) are alive., Conclusions: A survival rate over 90% can be achieved by conventional ventilatory measures in patients with IO<40. In our experience, children with IO>40 are rarely candidates to ECMO therapy because of the associated severe lung hypoplasia confirmed by necropsy studies.

Unlabelled: We analyize our experience in the management of the last consecutive 100 Hirschsprung's disease (HD) patients divided into two periods: 1992-1997 and 1998-2004, in order to find out differences in morbidity, mortality and outcome between them., Material and Methods: During this period, 72 males and 28 females were treated. Twelve had family history and five suffered from Down's syndrome. Information about clinical onset, need of stomas, surgical procedures, continence, outcome and mortality was recorded. We compared the results between the two groups with non-parametrics stadistics test., Results: 50% of patients were symptomatic in the newborn period and 25% of them needed some surgical procedures. Seventy four patients suffered from rectosigmoid forms, fourteen colic forms and twelve were total colonic HD (7 with small bowel extension). Hystochemistry was diagnostic in 98%. Nursing was effective in 47 cases. Differences in the need of stomas were found between the two periods: 30% during the first period and 6% during the second one (p<0,05). Twenty percent (20) of the patients suffered from enterocolitis (with no differences between both groups), and 13 of them still had enterocolitis episodes in spite of stomas or pull-through procedures. We performed 49 Swenson, 29 Soave, 14 transanal and 2 Lester-Martin procedures. The median age at definitive operation was smaller in the last period when compared to the first (p< 0.05). We found good results on continence in 86%, with no relation with definitive surgical procedure nor with the period of time studied., Conclusions: The younger age at definitive treatment, the performance of stomas and the increase of transanal procedures were the principal differences between the two groups.

Background: Cosmetic deformity and psychological repercusion are the main surgical indications in pectus excavatum. However cardiopulmonary function is subclinically abnormal in some patients. The goal of this paper is to demonstrate that heart response during physical activity improves after surgical correction of pectus excavatum., Patients and Methods: Fifty five patients were operated upon for pectus excavatum during the last 10 years at our institution. Baseline ecocardiography and isometric effort test (Handgrip) were preoperatively performed in 15 patients (11 males and 4 females) aged from 6 to 15 years (median 9,63). An increase below 12% in cardiac index was considered as positive. In those patients with positive tests, a new test was made 6 months after surgical correction. Pre and postoperative results were compared using non-parametric tests., Results: Baseline cardiac index values were normal in all cases (11). Four patients were not included, 2 because of no collaboration (aged 6 and 7 years) and 2 because difficult ecocardiography interpretation (both operated upon for congenital diaphragmatic hernia). Overall, preoperative test was negative in 6 patients and positive in the remaining 5. Postoperative test was made in these 5 patients, and all of them showed an improvement in cardiac index (medium 40,6%, range 25-70) when compared to preoperative values. There was statistically significant association between test values and patient ages., Conclusions: Baseline cardiac index values were normal in children with pectus excavatum, albeit 45,5% of them showed a limited response to exercise. Pectus excavatum repair improves these values, so physiopathological indication as well as cosmetic one should be considered specially in children.

Background: Midgut malrotation has a low incidence (0.5-1% at necropsies) and it is a rare symptomatic condition. Nevertheless it is often associated to clinical situations with a high morbility and mortality and it could complicate them. Our aim is to study the patients treated for abdominal wall defects and who have suffered complications due to non-treated malrotation at first surgery., Methods: We retrospectively studied the charts of patients diagnosed of abdominal wall defects in our hospital from 1993 to 2002. We reviewed the initial treatment, the associated morbility and any new surgical treatment needed., Results: During this 10-year period, 110 abdominal wall defects were managed: 56 congenital diaphragmatic hernias, 30 onphaloceles and 14 gastroschisis; overall 79 of these 100 patients survived and were included in this study. Eleven patients had symptoms due to bowel malrotation during the follow-up period. In 1 case the clinical picture had an acute onset as a volvulus that required extensive gut resection; another patient presented as a persistent gastrocutaneous fistula after removal of a gastrostomy tube; in the remaining 9, symptoms were always of classical intestinal obstruction. The malrotation was never treated during the initial surgical procedure for abdominal wall defect; later on, Ladd procedure was always the definitive treatment., Conclusions: Due to local conditions during first abdominal wall surgery that limit the evaluation of the malrotation, we must think about it when we find obstructive symptoms any time during life.

Background: Inflammatory pseudotumor (IPT) is the most frequent pulmonary mass in childhood. It is histologically benign but locally aggressive. Atelectasis and recurrent airway infections are the most frequent presenting findings. We present two children in whom first clinical signs were paraneoplasic syndromes., Material and Methods: Retrospective study of two cases of IPT treated in our clinic from 1998 to 2002. Age, clinical presentation, preoperative diagnosis, treatment, histological diagnosis and postoperative outcome were reviewed from clinical chart., Results: Case 1: 7 year old male with incidental diagnosis of superior right lobe IPT in routine study because of diabetes. The mass collapsed superior and median lobar arteries and compressed superior cava vein. Right pneumonectomy was necesary to complete removal of the mass. From immediate postoperatory the child became normoglycemic and is free of insulin. Case 2: 11 year old male with rheumatologic clinic consisting in hypertrophic osteoarthropathy, arthralgy and knees liquid lasting for two years. X-ray examination showed mediastinic mass. He underwent complete removal of a pulmonary mass. Few months after the operation the rheumatologic-like symptoms were resolved., Conclusions: IPT can present with paraneoplasic syndromes, although physiopathology is not well understood. In children with recurrent respiratory infections one diagnosis to take in mind is IPT, even more when clinic is associated with symptoms compatible with paraneoplasic syndrome.

Unlabelled: Oesophageal perforation (OP) requires prompt and vigorous treatment. In contrast with adult patients in whom surgical closure of perforation is preferred, non-operative treatment has been the usual approach in children. The present report aims at assessing whether this strategy stands the passage of time. We studied retrospectively the charts of patients treated at our institution for OP between 1991 and 2001. Between these years, we treated 19 episodes of OP in 17 patients aged 5.3 +/- 0,94 years. In 9 cases (4 lye burns, 3 oesophageal atresias, 1 bullous epidermolysis and 1 mucocutaneous candidiasis) OP occurred during dilatation of strictures. Foreign body extraction was the cause in 3 cases, and blunt trauma and sclerosis of varices were the causes in 2 cases each. The last child had multiple gastrointestinal perforation during treatment for leukaemia. Subcutaneous emphysema was seen in 7 instances, pneumomediastinum/pneumothorax in 14, pleural effusion in 9, dyspnoea in 9, severe thoracic pain in 1 and pericardial effusion in 1. The diagnosis was intraoperative in only 2 children but the symptoms and imaging signs prompted vigorous treatment within the first 24 hours in 15 instances. One or more pleural tubes were inserted in 11 cases and pericardial drainage was required once. Perforations closed without direct surgery in 18/19 episodes (16/17 children). Five gastrotomies and 2 jejunostomies were performed and several major abdominal operations were necessary to repair concurrent lesions in a child who sustained severe blunt abdominal trauma and in the one with leukaemic perforations. All these patients survive and all recovered oesophageal function although 2 with intractable lye structures ultimately required oesophageal replacement 6 and 10 months after OP. The only patient in whom direct approach for esophageal necrosis after variceal endosclerosis was unavoidable lost her organ and had a replacement after a successful porto-systemic shunt., Conclusions: Prompt and aggressive non-operative approach of oesophageal perforations in children allows survival and conservation of the organ and its function in most cases and should remain the first therapeutic choice at this age.

Background: Success in the treatment of vascular anomalies during infancy depends on an accurate early diagnosis and a correct therapeutic management. Current available resources can be divided into pharmacologic, endovascular, surgical, and laser. Results are variable, and complications should always be kept in mind., Methods: We reviewed all patients with vascular anomalies who suffered from major complications directly due to the treatment during the past five years., Results: 1. A patient with an upper lip hemangioma following treatment with interferon after no response to steroids. A long-term severe neutropoenia forced to an early surgical excision of the lesion. 2. A patient suffering from an orbitary hemangioma with severe exoftalmus was treated with interferon because of a steroid-resistance. A spastic diplegia due to interferon reverted after treatment was discontinued. 3. In a patient with a Blue-Rubber Bleb Nevus syndrome, extended percutaneous sclerosis was performed. She developed skin necrosis of the left leg and a permanent sciatic nerve paralysis. 4. In a patient with an arteriovenous malformation on the right leg and gluteous, a femoral artery endoprosthesis was placed because of a massive bleeding. She underwent a total excision of the malformation and developed a recurrent ischemia related to a femoral thrombosis. Finally a foot amputation was needed. 5. A patient with a Kaposi-like hemangio-endothelioma who was following a high-dose prolonged steroid therapy died because of a meningoencephalitis related to a severe immunosuppression., Conclusions: Treatment of vascular anomalies during childhood need an early accurate diagnosis. If a right therapeutic sequence is not performed, useful drugs can turn into inefficient or dangerous. Most treatment complications in these patients can be avoided.

Introduction: When primary anastomosis is not feasible in esophageal atresia esophageal replacement is one of the possible options. We report our experience with this approach in patients with long-gap esophageal atresia., Materials and Methods: From 1991 to 2002 we treated 50 children with esophageal atresia. Nine required esophageal replacement because of long-gaps. Six were boys and three girls. Six had isolated atresia, and three had regular atresia with TEF. Associated malformations in 8 patients were: cardiovascular (2), anorectal (2), urogenital (5), intestinal duplication (1), and costovertebral (1). The weight at birth ranged from 1.2 to 3.95 kg (median 2.3). Patients with type I atresia had immediate gastrostomy accompanied by esophagostomy in one. Esophageal anastomosis was impossible or failed in 3 patients with type III EA, who had fistula ligation and esophagostomy. Esophageal replacement was performed at a median age of 4.95 months (range 2.3 to 18), with a median weight of 5.33 (range 2.89 to 11.5 kg.). We used gastric pull-up in 2 cases and colonic transposition in 7 with isoperistaltic left transverse colon in retromediastinal-transhiatal (6) or restrosternal (1) position. Two patients had extramucosal piloromyotomy and 4 had piloroplasty., Results: All patients survive and have functional grafts. Postoperative complications were: pneumonia (1), wound dehiscence with evisceration (2) and salivary fístula (2) which closed spontaneously. In the long range, one patient was operated three times for hernia through the enlarged hiatus, one had one episode of intestinal subobstruction successfully managed with nasogastric aspiration and another one had dumping syndrome for several months. After a median follow-up of 3.15 years (range 1.6 to 6.9) all patients eat normal diets per os., Conclusions: Esophageal replacement for the treatment of infants with long-gap EA has been in our hands as good as any other option with 100% survival and good functional results. The operation can be safely performed in the first months provided that associated malformations are under control. However, like other options, this approach is not devoid of complications.

Introduction: Extensive aganglionosis (EA) that affects the total colon (including or not part of the small intestine), shows more diagnostic problems and it is associated to higher morbidity and mortality rates than the classic cases of Hirschsprung disease (ED). This study takes into account last years cases and their results in the medium and long term., Material and Methods: Between 1983 and 2000, 232 patients suffering ED have been treated. 15 out of those 232 patients showed EA. We took into consideration the diagnosis, surgical procedures, enterocolitis before and after the treatment, the surgical technique and the complications. We analyze the nutritional state and the long term result according to clinical bowel function scoring system continency Scale, considering the surgical possibilities depending on each case., Results: Two patients died due to septics complications, one of them during the neonatal period and the other one at the age of 3. Two other cases became possible candidates to an intestinal transplantation due to the extension of their disease. Five out of the 11 left patients had more than 30 cm of small intestine involved, and another five had involved less than 30 cm from ileocecal valve. The last case showed a total colonic aganglionosis with associated intestinal neuronal displasia. One case was associated with S. Ondine and another one with a 21 triosomia. Two of the patients were brothers and another patient has got a brother suffering from rectosigmoid HD. The surgical techniques used with 13 were: 8 cases using Lester Martin modification (one of them needed redo procedure because of anastomosis leak and perineal fistula), 4 Swenson procedure and one patient was treated by Ziegler's miectomy with prolongated miotomy. Nine of the patients mentioned before, lead a quite life (five L, Martin, 4 Swenson); 2 patients suffering frequent fecal retention crisis. 5 out of the 11 reviewed cases, show a weight and height p > or = 50, 2 below p50, 2 below p25, and 2 under p3., Conclusion: EA represents a small percentage of Hirschsprung disease. Although Martin modification is the most widely employed technique in cases of distal ileal involvement, Swenson procedure has provided good results in the long term follow-up with few complications. Intestinal transplantation may be the only choice for long term survival in patients with near total intestinal aganglionosis. Continence improves in the follow-up but few times is normal. Nutritional state should be-controlled by a specialized team.