Health-related quality of life in pediatric intestinal transplantation.

To determine HRQOL after pediatric intestinal transplantation. Thirty-four IT survivors from 1999 to 2012 were asked to complete age-specific HRQOL non-disease-specific questionnaires: TAPQOL (0-4 yr), KINDL-R (5-7 yr; 8-12 yr; 13-17 yr), and SF-36v2 (>18 yr), all validated with Spanish population. Primary caregiver completed a SF-36 questionnaire and CBI. Thirty-one participants were included. Median age was 10.2 yr (1-29) and time after transplant 4.4 yr (0-13). Overall patient scores were 78.2 ± 10.6 (n = 8), 83.3 ± 9.7 (n = 6), 72.2 ± 9.21 (n = 6), 80.5 ± 12.4 (n = 7), and 82.2 ± 12.4 (n = 4) for each age group. Highest scores were obtained for vitality (group I), self-esteem (group IV), and physical and social functioning and emotions (group V). Lowest scores were obtained in appetite and behavior (I), family and school (III), and chronic disease perception (III, IV). No significant differences were found between caregivers and their children. CBI showed stress in 52%. SF-36 for caregivers was lower than general population. No significant differences were found depending on relevant clinical and sociodemographic data. HRQOL was acceptable and improved with age and time since transplantation. Parents had a slighter own QOL and worse perception of health than their children. When successful, intestinal transplantation allows a normal life in most patients and can be offered as an attractive option.

Preservation of the native spleen in multivisceral transplantation.

The native spleen is usually removed in patients undergoing MTV. The consequential asplenic state is associated with a high risk of sepsis, especially in immunosuppressed children. In contrast, the inclusion of an allogeneic spleen in multivisceral grafts has been associated with a high incidence of GVHD. We propose an alternative technique for patients undergoing MTV, consisting of the preservation of the native spleen. This approach avoids the additional risk of infection that characterizes the asplenic state without the detrimental side effects of the allogeneic spleen.

[Technical aspects of experimental intestinal transplant]. / Aspectos técnicos del trasplante intestinal experimental.

PURPOSE: Our objective is to analyze the variables that influence the outcome of Small Bowel Transplantation (SBT) in rats in an experimental microsurgery program. The surgical technique and perioperative care are described in detail. METHODS: Retrospective study of the SBT in rats conducted in our experimental surgery laboratory from 2002 to 2010. The animals were divided into group A (those who survived more than 48 hours) and group B (those who died earlier without justificable cause). We compared in both groups: number of transplants performed by the surgeon, warm ischemia time, cold ischemia time and duration of the procedure. RESULTS: Five surgeons with different degrees of microsurgical training participated in the study. A total of 521 SBT were performed with an overall survival of 48%. The first successful transplant was performed after a median of 46 (25-68) transplants. Total procedure time (3.5 vs 2.9 hours) and warm ischemia time (51 vs 35 minutes) were higher in group B (p < 0.05). DISCUSSION: The number of transplants required for learning the technique is high. However, survival is acceptable when the time needed for vascular anastomosis is reduced. The SBT in rats is a valuable model for surgical training and research of the phenomena related to SBT.

Selective portal embolization combined with chemotherapy treatment in non-resectable liver tumors reduces tumor volume and generates contralateral compensating hypertrophy allowing for a full resection while avoiding liver transplantation. / La embolización portal selectiva coadyuvante al tratamiento quimioterápico en tumores hepáticos no resecables produce una reducción tumoral e hipertrofia compensadora contralateral que permite una resección completa evitando el trasplante hepático.

INTRODUCTION: Non-resectable liver tumors are one of the most frequent indications of pediatric liver transplantation. However, the usefulness of interventional techniques is still to be exploited. This is the case of a patient with a non-resectable liver sarcoma proposed for liver transplantation, which could be avoided as the tumor was fully resected following selective portal embolization combined with chemotherapy treatment. CLINICAL CASE: The patient was a 10-year-old female with a large hepatic mass compatible with undifferentiated PRETEXT III sarcoma treated according to the EpSSG RMS2005 protocol for high-risk tumors, but without achieving any reduction in volume. Given the risk of developing a small-for-size syndrome following a potential resection, a right portal embolization was performed in order to induce left hemi-liver hypertrophy. No response was observed after two months, so the patient was included on the liver transplantation list. However, one month later, a significant reduction in mass volume was noted at a control CT-scan, which meant the tumor could be resected while avoiding transplantation. The one-year follow-up was uneventful. DISCUSSSION: Interventional techniques should be considered as the main therapeutic option in non-resectable liver tumors, since they could potentially avoid transplantation in selected patients.

INTRODUCCION: Los tumores hepáticos irresecables figuran entre las indicaciones más frecuentes del trasplante hepático pediátrico. Sin embargo, las técnicas intervencionistas suponen un campo terapéutico con utilidades aún por explotar. Presentamos el caso de una paciente con un sarcoma hepático irresecable propuesta para trasplante hepático, que pudo evitarlo al ser completamente resecado tras realizar una embolización portal selectiva coadyuvante al tratamiento quimioterápico. CASO CLINICO: Paciente mujer de 10 años con masa hepática de gran tamaño compatible con sarcoma indiferenciado PRETEXT III tratado según el protocolo EpSSG RMS2005 para tumores de alto riesgo sin conseguir reducción del mismo. Debido al riesgo de desarrollar un síndrome small-for-size tras una eventual resección, se llevó a cabo una embolización portal derecha para inducir la hipertrofia del hemi-hígado izquierdo. Tras dos meses no se evidenció respuesta alguna, por lo que la paciente fue incluida en lista de trasplante hepático. Sin embargo, un mes después se observó una clara disminución de la masa en un TC de control y la resección pudo realizarse con éxito evitando así el trasplante. Tras un año de seguimiento, la paciente no presentó ningún evento clínico de interés. DISCUSION: Las técnicas intervencionistas deben ser consideradas como una opción terapéutica en tumores hepáticos irresecables, ya que podrían evitar el trasplante en pacientes seleccionados.

[Chronic idiopathic intestinal pseudoobstruction and Berdon syndrome: still a diagnostic and therapeutic challenge for the pediatric surgeon]. / Pseudoobstrucción intestinal idiopática crónica y síndrome de berdon: todavía un desafío diagnóstico y terapéutico para el cirujano pediátrico.

AIM/BACKGROUND: Chronic Intestinal Pseudo Obstruction (CIPO) and Berdon Syndrome (BS) are motility disorders with still unclear pathophysiology, and challenging diagnosis and management. Patient and methods. 26 patients (8M/18F) treated of CIPO (21) or BS (5) at our institution between 1982-2009 were retrospectively reviewed and clinical, diagnostic, therapeutic and follow-up data were analyzed. RESULTS: 77% had a neonatal onset by the 3rd month of life (5 had a prenatal diagnosis of megacystis). Abdominal distension (87%), recurrent suboclusive episodes (70%) and malnourishment (60%) were the main symptoms followed by vomits, chronic diarrhea, constipation and dysfagia. The urinary tract was involved in 12 patients (46%): 8 had megacystis, 8 had vesicoureteral reflux. Arrythmias were seen in 2, deafness in 1, hydrocephalia in 1 and malrotation in 5. Radiological studies were consistent for CIPO in all of them: the disease was limited to the esophagus in 1, 3 had segmentary involvement of the small bowel, 6 had microcolon, and the rest had all the digestive tract involved. Anorectal manometry ruled out aganglionosis in 12, esophageal manometry showed aperistalsis in 9 and antro-duodenal manometry confirmed the diagnosis in 9 (visceral myopathy in 4 y neuropathy in 5). Rectal biopsies (16) and muscular biopsies (5) were normal in all of them. Full thickness biopsies (in 18, after surgery) showed myopathy in 12 and neuropathy in 6. Prokinetics and antibiotics for bacterial overgrowth were employed in 100%, 17 required long-time parenteral nutrition (PN), 21 required surgery and 7 were transplanted (4 isolated small bowel, 3 multivisceral). Symptoms improved in 9/15 with an ileostomy. 19 weaned from PN. After a median follow-up of 7.9 years (range 5m-17a), 3 were lost, 17/23 patients are alive and only 2 on home PN. Six died, 3 after being transplanted. CONCLUSIONS: CIPO and/or BS have a wide clinical spectrum and a complex diagnosis; however, the knowledge of the disease and an appropriate treatment, generally aggressive, allows the patients with CIPO to enjoy a long-term acceptable quality of life.

[Need of hepatic bipartition or split in the transplant in children]. / Necesidad de la bipartición hepática o split en el trasplante en niños.

AIM: To analyze the benefits of Split (for adult and for child) in liver transplantation. PATIENT/METHODS: 1) Analysis of the waiting list mortality estimated on 228 inclusions for transplant since January 2004 to December 2008.2) Impact of the variant techniques (living-related donor and split) on the waiting list mortality in our patients. 3) Analysis of the outcome of 33 split livers which allowed to perform 66 transplants (1994-2008). RESULTS: Estimated as number of patients by 1,000 candidates by year of exposure, the waiting list mortality was 110 in children older than 5 year old, 180 in children from 2 to 5 year-old, 90 in children between 1 and 2 year-old and 510 in younger than 1 year (p<0.05 for the last group). 36/66 split grafts were implanted by our group. Five grafts were lost, 3 due to retransplantation and 2 due to death. Overall patient/graft survival alter 10 years of follow-up was 94.5% and 85.1%, respectively. The rest of the grafts (n=30), were used in other hospitals, and 4 were lost in the early postoperative period. Since the beginning of the study, 85.4% of children between 1 and 2 years, received a living-donor or a split graft, as only 59.9% in the younger than 1 year-old group. CONCLUSION: Our results absolutely justify the ethics of split liver transplantation for an adult and a child. Despite other factors, the benefits of the variant techniques in the 1-2 year-old group are obvious. Up to 60% optimization with these techniques in children younger than 1 year would not be yet enough in order to decrease the mortality waiting list down to that of the rest of the groups.

[Liver bipartition as an alternative to the transplant]. / Bipartición hepática como alternativa en el trasplante.

AIM: Liver pediatric transplantation finds in the lack of donors its main limitation. An alternative in those cases is split liver grafts from bigger donors. PATIENTS AND METHOD: We performed a retrospective study of 56 hepatic split transplants performed between 1994 and 2007. Twenty-nine children were transplanted with a median age and weight of 1.8 years old (0.3-9) and 9.7 kg (6.2-23). In 16 cases (53.3%) liver transplant was performed in emergency situation. In one patient we performed a combined transplant (liver-kidney) and in another patient it was a second transplant due to primary graft failure after receiving an hepatointestinal allograft. Type of grafts used were: lateral left segment (n=26), extended lateral left segment (n=1) and extended right liver (n=3). Median donor age and weight were 20 years old (8-44) and 60 kg (24-80). We studied patient and graft survival (Kaplan Meier), perioperative factors, complications and net rate of early complications in adults recipients. RESULTS: Patient survival was 96.7% after 6 months, 1 year, 5 years and 10 years. Id for grafs 86.7%. Two grafts were lost due to arterial thrombosis, one due to primary non function and another due to recipient death secondary to a sepsis. Five children had major biliary complications and 2 of them developed multiple intrahepatic stenoses, one of them being on waiting list for retransplant. Early graft lost (retransplant or death before leaving the hospital) occurred in 4 out of the 25 grafts transplanted in other centers (25 adults, 1 kid); all of them occurred in the initial period (1994-2001). CONCLUSIONS: Even though it is clearly documented that benefit of transplant (measured in years of life won) is very good after split transplantation, nowadays criteria for organ allocation in Spain do not allow a more extensive diffusion of this technique and it is confined to urgent transplant. Even in those cases, results after split transplantation are excellent. Without this possibility our pretransplant mortality would be much higher.

Variant techniques for liver transplantation in pediatric programs.

INTRODUCTION: Several variant techniques have been developed as alternatives to whole liver transplantation to improve size matching, timing, or simply to increase the pool of donors. The aim of this study was to assess the requirements of these techniques and their outcomes in a pediatric transplant program. PATIENTS AND METHOD: A retrospective analysis of children on the waiting list in the last 4 years was carried out. Data of patients who died while on the waiting list (WL) were recorded. Transplanted patients were divided according to the type of graft: whole liver, split, living donor and reduced liver. The analyzed outcome variables were: age, weight, UNOS status, cause of liver failure, complications and graft and patient survival. Comparisons between types of graft were performed by using Kaplan-Meier, log-rank, chi (2) and Kruskal-Wallis tests. RESULTS: During the period studied, 116 children were listed for liver transplantation. Of these 116 children, nine (7.7 %) died after a mean period of 40.5 (5-175) days waiting for a suitable graft. Their median age at inclusion was 214 (35-1607) days, and median weight was 7.2 (12.3-3.6) kg. The cause of liver failure in this group was: 1 hemochromatosis, 1 hepatoblastoma, 2 biliary atresia, 2 acute liver failure, 2 primary non-function (PNF) and 1 chronic rejection. Liver transplantation was performed in 103 children: 25 (24 %) whole livers, 17 (16.5 %) split, 29 (28 %) living donor, 32 (31 %) reduced and 4 remain on the waiting list. Recipient age and weight were significantly lower in those receiving split and living donor than in those who given whole livers. Patient and graft survival were similar in all groups although there was a trend to lower graft survival in patients receiving whole livers. More than 50 % of patients with UNOS status I received a split graft and 5/6 children with hepatoblastoma underwent living donor transplantation. There were no differences in the rate of acute vascular complications, but long-term biliary complications were more frequent in split and living donor grafts. CONCLUSIONS: As long as the goal of zero mortality for children on the waiting list is not achieved, variant techniques will be necessary in pediatric liver transplantation programs. Split and living donor were employed mostly to treat younger children and particularly those with a higher UNOS status. Children with tumors were treated mainly with living donor grafts. Variant techniques, which are absolutely necessary in a pediatric program, need to be improved in order to avoid long-term biliary complications.

Long-term results of the treatment of total colonic aganglionosis with two different techniques.

AIM: Aim of this study was to assess the long-term results of the treatment of total colonic aganglionosis (TCA) with 2 different techniques in terms of growth, continence and quality of life (QOL). PATIENTS AND METHODS: Forty-one patients treated for TCA between 1972 and 2007 were reviewed retrospectively with special attention paid to the length of aganglionosis, complications, growth, continence and QOL. Until 1992, patients underwent subtotal colonic resection and side-to-side ileosigmoid anastomosis (modified Martin). Since 1992, straight ileo-anal pull-through was preferred. At the end of follow-up (median 18 years, range 1-35), the height and weight, continence and QOL (scoring feeding habits, school/work performance, family life and professional development) were assessed by clinical visit or phone interview. RESULTS: Twenty-eight patients were male and 13 female. Six had total intestinal aganglionosis and were excluded from this review together with the 2 who died before definitive treatment. The 33/41 persons in whom aganglionosis had involved less than 50 cm above the ileocecal valve and who had been considered suitable for the reestablishment of transanal fecal flow were included. Eighteen children underwent a modified Martin and 15 straight ileo-anal pull-through. Postoperative intestinal obstruction occurred in 4 cases, prolapse and prolonged TPN requirement in 2, and wound disruption and fistula in 1. Thirteen patients (39 %) had postoperative enterocolitis. Two children died after operation (1 wound disruption with sepsis and 1 pneumonia). Out of 31 survivors, 57 % and 53 % were > p50 with regard to height and weight whereas only 15 % and 19 % were

[Morbility and mortality associated with prosthetic patch in congenital diaphragmatic hernia]. / Morbimortalidad en pacientes con parche protésico para el cierre de la hernia diafragmática congénita.

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.

Surgery of liver tumors in children in the last 15 years.

AIM: Aim of the study was to review our experience in the management of liver tumors in children over the last 15 years. PATIENTS AND METHODS: A cohort of 78 children with liver tumors managed in our institution between 1991 and 2006 was retrospectively reviewed. There were 45 males and 33 females with a mean age of 32 +/- 41 months at diagnosis. Most tumors were malignant (n = 57); the most frequently occurring tumor was hepatoblastoma (n = 47), followed by hepatocarcinoma (n = 5), sarcoma (n = 4), and lymphoma (n = 1). Vascular tumors (n = 12) predominated among the benign tumors followed by mesenchymal hamartoma (n = 4), focal nodular hyperplasia (n = 3), adenoma (n = 1), and inflammatory pseudotumor (n = 1). We reviewed the epidemiologic features, clinical presentation, diagnosis, treatment and outcomes. We employed MRI and angio-CT for SIOPEL PRETEXT staging and selected the management accordingly for malignant tumors. We analyzed the long-term survival using Kaplan-Meier curves. RESULTS: Benign tumors had an excellent outcome with both medical or surgical management. Of the malignant tumors 4 were PRETEXT I and were treated by left lateral segmentectomy with 100 % survival; 20 were PRETEXT II (12 left and 8 right lobe) and were treated by lobectomy of the corresponding side, except for 1 case which required OLT (90 % survival); 9 children had PRETEXT III tumors requiring trisegmentectomy or extended lobectomies with OLT in 1 case (77.7 % survival). Fourteen children had PRETEXT IV tumors: 10 received OLT and 9 of them are still alive (64.2 % survival). Overall survival was 80.8 %, and actuarial survival at 6 years was 82.2 %. Other malignant tumors had variable results. CONCLUSIONS: Outcomes have improved much in the last years. Surgical removal is necessary in most cases. Transplantation is a very useful adjunct. Treatment of these tumors should be concentrated in centers with expertise.

Sternal cleft associated with enterogenous cyst treated during the newborn period.

We present the case of a newborn with sternal cleft (SC) and presternal enterogenous cyst operated on during the neonatal period. SC is an uncommon congenital malformation of the thoracic wall which can occur as an isolated form or in association with other malformations. To our knowledge, the presence of SC and enterogenous cyst has not been described to date. Early surgical repair of SC gives good aesthetic and functional results and is usually the preferred approach.

[Early surgical treatment in nasal tip hemangiomas: 36 cases review]. / Tratamiento quirúrgico precoz de los hemangiomas nasales: revisión de 36 casos.

BACKGROUND: Hemangiomas are the most common benign tumours in infancy. Its trophoblastic origin is now confirmed and it would explain their increasing incidence that runs parallel to the more frequent use of fertilization in vitro techniques and the high rates of prematurity and twinning. It is not a coincidence that they appear more often in the face and its location is related to the merging lines of the facial embryonal buds. Nose tip, upper-eyelid and orbital regions are special areas that need a different approach in each case. Over the last years we changed our therapy protocol for these tumours, in an attempt to decrease the psychological, social and scholar impact in children. PATIENTS AND METHODS: During the last 20 years, 36 patients with nasal tip hemangiomas underwent surgical treatment in our institution. We divided them into two groups, those treated between 1985 and 1992 (n=l1) and those treated between 1992 and 2005 (n=25). Traditionally, conservative management including close observation, corticosteroid therapy and finally surgical treatment of the sequelae, was proposed. Late surgical treatment was reserved for incompletely regressed or unsuccessfully treated facial tumours. Our more recent approach involves early surgical excision, in order to avoid psychological distress and cosmetics defects. Age at diagnosis, appearance at that moment, sort of treatment and time of surgical procedure were recorded to evaluate long-term results. RESULTS: In this paper we report our series of 36 patients, consisting of 26 females and 10 males, with a mean age of 5 +/- 2 months at the first visit. In 11 patients from the first period (1985-1992), corticosteroid therapy was applied waiting the spontaneous regression and they finally underwent delayed surgical treatment at a mean age of 8 years. In the remaining 25 patients treated in the second period, early surgical excision was carried out with a mean age of 3 years and before school age. Indications for corticosteroid therapy include uncontrolled growth and complications (ulceration). CONCLUSIONS: The approach to management of nasal tip hemangiomas should be individualized and must take into account their depth, location, rate of involution and functional disturbance. Children age as well as psychological problems arising from the presence of proliferative hemangioma must be considered when deciding a surgical approach. Upon the high rates of scholar failure in children with deforming hemangiomas, the lack of response to medical therapy and the need of surgical treatment in all cases, our policy now it is to attempt surgical excision and reconstruction before school age. Based on data reported and on our own experience, we consider that very early surgical treatment improves quality of life of our patients and their families.

[What can we do when a esophagocoloplasty fails?]. / Qué hacer cuando fracasa una esofagocoloplastia?*.

BACKGROUND: Postoperative complications of esophageal replacement are potentially severe, and they can even make impossible the digestive continuity. However, several surgical options have been used for such situations. The aim of the study is to assess the early and late results in patients who have been managed or referred to our department for this problem. PATIENTS AND METHODS: We reviewed 8 patients operated between 1975 and 2005, four of them were referred from other hospitals. The ranging in age was from 4 months and 23 years at the moment of the first colonic replacement, and who required a second plasty because of intra or postoperative complications. The esophagocoloplasty was retrosternal in 6 cases and transhiatal in 2, with a left colon graft in 6, ileocolonic in I and right colon in the last one. The initial diagnosis was traqueoesophageal fistula in 6 (type III in 3, type I in 2 and IV in 1), caustic injury in 1 and herpetic esophagitis in 1. Complications requiring reoperation were stricture of the cervical esophagococolic anastomosis because of postoperative dehiscence (n=4), perioperative deficient graft vascularisation (n=2), graft necrosis (n= 1) and symptomatic gastric-colic reflux (n=l). RESULTS: The deficient graft irrigation was detected and managed during the surgical procedure in 2 cases, left colonic graft was remplaced by a gastric tube and the right colon, respectively, with excellent results. Repeated endoscopic dilatations (n=4) as well as surgical revision with resection and reanastomosis of the stenotic segment (n=2), did not suffice in children with severe strictures (100%). A second plasty was tried in 2 patients: a failed microsurgical sigmoid graft in one of them, and a presternal esophagocoloplasty with a left colonic graft in the other one. It was impossible to perform a new plasty in two chidren, because of the severe mediastinal fibrosis. One patient was referred with severe gastro-colic reflux and the cologastric anastomosis and the gastrostomy were refashioned. Another patient with graft necrosis required total resection of the coloplasty and a new esophagostomy and gastrostomy. 50% of the patients recovered digestive continuity, and nowadays three of them eat normally. The fourth one died after several years because of an Guillain Barré syndrome. Four patients are still waiting for future attempts of esophageal substitution. CONCLUSIONS: There are surgical options to reestablish the digestive continuity whenever the initial esophageal replacement fails. Severe postoperative strictures do not dissapear with endoscopic dilatations or stenotic resection and reanastomosis, but they usually require a new graft in another less injured place. Poor vascularisation of the graft can be prevented, giving the best solution for each patient, and at minimal suspicion of ischemia, consider another plasty or access during the operation.

[Pediatric patient in adult age. Long-terms results of esophageal replacement]. / El paciente pediátrico en la edad adulta: revisión a largo plazo de los resultados de la sustitución esofágica.

BACKGROUND: Esophagocoloplasty is one of the most used procedures for esophageal replacement in children. Considering high life expectancy in these patients, long-term results must be considered when evaluating this technique. The aim of our study is to evaluate quality of life of adult patients who underwent surgery at pediatric age. PATIENTS AND METHODS: We report a retrospective study of 99 patients who underwent esophageal replacement in our institution between 1966 and 2006. Eight of them have died and 63 out of the remaining 91 are over 18 years now and represent our study serie. Long-term results and actual situation of those patients, considering psychological, physic and social aspects, were evaluated through clinical review and telephonic interview. Karnofsky index was applied to mesure functional ability from 0-100% (bad, medium, good-excellent) according to the answers the patients gave to our questions. We also recorded their health personal experience and subjective evaluation of their quality of life. RESULTS: Sixty-three patients were reviewed (43 males and 20 females) with a mean age of 4.3 +/- 3.4 D.S. Mean follow-up time was 29.6 +/-7.7 years. Indications for esophageal replacement were as follows: caustication (n = 32), type III esophageal atresia (n = 15), type I AE (n = 13) and others (n = 3). In 48 patients the graft was placed in retroestenal position and in 15 cases retromediastic location was used. Postoperative period was uneventful in 44% of the patients, being the most frequent early complications in the remaining, cervical leakage and stenosis. Long-term, 56,8% did not have any sequelae, 28.5% required further surgery and the remaining 43.13% presented the following complications: symptomatic graft reflux (22), scoliosis and thoracic asymmetry (12), colonic redundancy or cervical diverticulum (7), food impaction (6) and failure to thrive (5). Only one 38 year old patient does not have intestinal tract continuity nowadays. Thirty-one patients have a Karnofsky index > or = 80-100%, being considered healthy and able to have a normal activity. Eighteen patients are included between 40-80%, being the most frequent limitation the need of medication to avoid reflux, backache and occasional episodes of food impact. Only 2 patients have Karnofsky index inferior to 40%. None of them are under 20%. CONCLUSIONS: Esophagocoloplasty allows restoration of intestinal tract continuity in almost all cases and the mortality of this procedure has decreased over time. Even though some risks are still remarkable, it offers long-term good results with little repercussion on functional ability in adult age. Most of the patients consider themselves healthy and enjoy an acceptable quality of life.

Liver transplantation for malignant tumours in children.

OBJECTIVE: The object of this study was to analyse our results with liver transplantation (LTX) for primitive malignant unresectable liver tumours in children and to discuss the controversial indications, based on our experience. METHODS/PATIENTS: We report on 12 patients, aged 6 months to 14 years, with hepatic malignant tumours: 11 with hepatoblastoma and 1 with fibrolamellar hepatocelullar carcinoma without cirrhosis. LTX was the primary treatment in 10 patients (PRETEXT IV or any grade, if there was extension to the retrohepatic vena cava, 3 hepatic veins or portal vein) and a rescue therapy after recurrence for 1 and for persistence of unresectable macroscopic residuals in 2 patients. One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We used entire liver (n = 5), left lateral segment from cadaveric donor (n = 3), living related donor (n = 3; 2 segments II-III and 1 right lobe) and left lateral segment from split liver (n = 1). All children received chemotherapy prior and post transplantation following the SIOPEL protocol. We analysed procedure tolerance, survival, recurrence rate, disease-free period and risk factors for adverse evolution. RESULTS: All patients overcame LTX and no early graft loss was recorded. Two cases died because of tumour relapse, 1 after primary LTX and 1 after rescue LTX (survival rate of both groups, 90% and 50%). Graft and patient survival rates at 1 year, 3 years, 5 years and 14 years were 91%, 91%, 82% and 82% respectively. The boy who presented with lung metastases developed new ones one year after LTX that were removed and he is currently free of disease. The disease-free period has a probability at 1, 3 and 5 years of 91%, 75% and 75%, respectively. Tumour tissue persistence was the only risk factor for an adverse clinical course in our series. CONCLUSIONS: LTX is a reasonable therapeutic approach for unresectable malignant liver tumours, providing outcomes comparable to those for resectable tumours. Results obtained with LTX are better when it is used as a primary treatment than when used as a rescue procedure. Proper staging and early referral to centres with enough expertise optimise the results. LTX for patients with lung metastases could be a controversial option. Living related-donor transplantation is an excellent alternative to avoid disease progression while on the waiting list for cadaveric donors.

[Venous malformations. Diagnosis and treatment during the childhood]. / Malformaciones venosas. Importancia de su diagnóstico y tratamiento en la infancia.

INTRODUCTION: Congenital venous malformations are the most common slow-flow vascular abnormalities during childhood. Although they are usually present at birth, clinical evidence often appear during late childhood. This type of malformations do not disappear, and they grow progressively during years. PATIENTS AND METHODS: We present a retrospective study based on the medical records of 72 patients treated in our Department during the last 15 years. Patients' age at the diagnosis was 5 days to 26 years. Male-female relationship was 2:1. The most frequent locations were Upper and lower extremities, followed by head and neck. We do not include in this study those patients with mixed lesions involving CNS or abdomen. RESULTS: Main symptoms were: pain, 60%; thrombophlebitis, growing size of the extremities, dermis lesions, osseous dystrophy and chronic coagulopathy. Symptoms worsening was related to trauma, infections and hormonal changes. Clinical course was the clue for the diagnosis. Eco-doppler was useful to study the flow of the lesion. MRI was employed to determinate the limits of the lesion. We did not perform arteriography, and phlebography was used as a diagnostic and therapeutic tool. Plain X-rays showed phleboliths in most of patients and osseous changes in a group of six patients. Treatment was conservative and palliative using elastic stockings, pain therapy and thromboembolism prophylaxis. This was the only treatment in irresectable lesions and in those cases without functional abnormalities. Surgery and sclerotherapy were the main curative treatments. Sclerotherapy was employed in cases of intramuscular and in delimited lesions. Ethanol and Ethibloc (Sodic Amidotrozoate) were the sclerosant agents. We performed surgery in cases of cutaneous complications, localized muscular lesions and in cases of recurrences after sclerotherapy. Ablative surgery was followed by reconstructive attempts using cutaneous and dermal artificial grafts. CONCLUSIONS: Congenital venous malformations must be diagnosed and treated during childhood. Conservative and resective treatments are useful in different cases. Accurate diagnosis and treatment improve long term results.

[Double toe to hand transfer in children with symbrachydactyly]. / Doble transferencia de dedo de pie a la mano en niño con simbraquidactilia.

INTRODUCTION: Symbrachydactyly is fairly frequent congenital malformation that appears during first weeks of pregnancy. The range of clinical presentations goes from hypoplastic fingers to true agenesis. Although it usually appears in a random fashion, sometimes it is associated to other systemic malformations. PATIENTS AND METHODS: The aim of this study is to check the functional, cosmetic and psychological results of those patients affected of grade IV symbrachydactyly treated using a double second toe transfer. The method employed is a retrospective study of the clinical records from 6 patients (5 males and 1 female) operated in our Department during the last five years. The follow up period was from 3 months to five years. RESULTS: Transfer from the second toe to the first ray was performed during the first surgical period, while transfer to the fourth ray was done in a second procedure. The mean age for the first operation was 19 months, and 30 months for the second one. There were no flap failure or major surgical complications. Tenolysis and tenoplasty was done in two patients to improve flap function and position. Motion rehabilitation was installed during the postoperative period. Functional, opposing pinch, and cosmetic results were satisfactory. Parents "and patients" psychological acceptance was also good. No walk disturbance was observed. CONCLUSIONS: Double second toe to hand transfer is a good option for the symbrachydactyly treatment in cases of thumb agenesis. Rehabilitation is the key clue for functional recovery. Surgery improves social and family relationships.

[Surgical management of cutaneous and musculoskeletal complications in fulminant sepsis]. / Tratamiento quirúrgico de las complicaciones cutáneas y musculoesqueléticas de los pacientes con sepsis.

INTRODUCTION: Surgical management of purpura fulminans is required as in the acute as in the sequelae period. A retrospective case review of these sequelae, procedures which have been performed in the different moments and the outcomes was undertaken. METHODS AND MATERIALS: We reviewed retrospectively 13 patients treated in our hospital from 1993 to 2003. 8 males and 5 females, with a median age of 2 years old (4 days-14 years) were managed. 70% were meningococcical. The most common areas were low extremities (92%), upper extremities (76%), lips (n=1), nose (n=1) and genitalia (n=1). Most of cases were bilateral (n=11) although two patients presented isolated affectation in one of the extremities. The procedures were classificated depending on the surgical moment. Clinical follow-up was attempted on all surgical patients; we evaluated the aesthetic, functional and psychosocial outcomes. RESULTS: One child died along the first 24 hours; 11 required early surgery and all of them required late interventions. 50% in the serie had necrosis over 15%. In Intensive Care Unit, it was necessary to do 6 fasciotomies (extirpating muscle in 2) and escharectomies and debridements in 8. Early amputations were made in 2 patients: both of them required ampliation in the following days. The cutaneous defects were covered with skin allografts and artificial dermis. During the sequelae period, 8 amputations were necessary (the four extremities in one case). Other procedures were coverage with autologous skin grafting (n=7), Zplasties, Wplasties (n=5), myocutaneous flaps (n=3), release of contractures (n=3) and osseus resection (n=5). There were skeletal growth disturbances which were managed with osteotomies (n=3), realineations(n=3), application of Ilizarov (n=2) and Hoffman (n=2) frames; Nowadays, 6 patients have a complete independent functionning with the use of orthotics (n=3) and prosthetics (n=3) CONCLUSION: The pediatric surgeon has an important role in management and prevention of complications in patients with purpura fuminans. Urgent actuation and close follow-up decreases the morbidity; furthermore, the early reparation of sequelae improves their adaptation in school, in the family and in the society despite the physical disability.

[Treatment protocol for extravasation lesions]. / Protocolo de tratamiento de heridas por extravasación.

INTRODUCTION: Morbidity of tissue extravasations is far of being properly considered in our hospitals. The increased survival rate in very low prematures and severe oncological patients has also produced an increase in the incidence of this complication, that in the most of the cases are not agressively treated in order to minimize the extravasation consequences. We have reviewed our experience in the treatment of these lessions using either injection and saline flushing of the subcutaneous (Gault tissue protocol, 1993) or coverage with artificial dermis in cases with irreversible necrosis. PATIENTS AND METHODS: Between 1998 and 2004, 15 patients with a median age of 3 years (range 3 months- 12 years) were treated because of extravasation injuries. The extravasated solutions were: parenteral nutrition (7), calcium salts (4) and doxorrubicine (4). Ten patients (66%) were treated inmediately according to the Gault protocol. Seven out of the 10 did not suffer any cutaneous loss, and the remaining 3 had only minimal lessions. Debridement of inviable tissue and coverage with articial dermis after 2 or 3 weeks was made in 5 patients (33%). All of them obtained functional and esthetic satisfactory outcomes. CONCLUSIONS: Extravasation injuries must be early evaluated by the surgeon and treated inmediately using saline instilation and subcutaneous flushing. In severe cases with total skin necrosis, artificial dermis proporcionates good esthetic and functional results, similar to other complicated techniques, which are nor indicated in prematures or critical oncological patients.