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Introduction: L'hypoglycemie hyperinsulinique persistante neonatale et infantile (PNHH) est une entite rare qui doit etre elucidee, mais qui est souvent associee a des lesions severes chez les patients pediatriques. Le but de cette etude est de presenter notre strategie chirurgicale habituelle pour cette maladie, basee sur nos constatations pathologiques et notre experience clinique. Materiel et Methodes: Ceci est une etude retrospective de 29 patients traites medicalement et chirurgicalement dans notre centre. Chez 15 patients, la morphologie, la morphometrie et l'immunohistochimie pour l'insuline, la somatostatine et le glucagon ont ete realises et il a ete possible d'etablir qu'il s'agissait d'une forme focale ou diffuse. Resultat: Chez 29 patients etudies, 25 etaient diagnostiques avant un an et 4 entre la premiere et la seconde annee de vie. Parmi les 25 patients, un est decede 7 heures apres l'accouchement. Douze patients ont recus un traitement medical seul: un est decede a 45 jours de vie et les autres ont un devenir correct. Les autres 12 patients ont ete traites chirurgicalement. Chez 2 de ceux-ci, un adenome etait observe et enleve et les patients gueris. Une pancreatectomie sub-totale etait realisee chez les 10 autres. (Un cas etait normal et gueri et les 9 autres avaient une lesion de type diffuse). Parmi les 9 patients avec une forme diffuse, 4 sont morts, 3 sont gueris et 2 ont necessite une reintervention chirurgicale. Parmi les 4 patients diagnostiques plus tard, 3 ont subi une intervention chirurgicale, 2 avec un adenome et 1 avec une forme diffuse. Les autres ont recu un traitement medical seul. Conclusion: Nous donnons regulierement un traitement medical pour tous les types et formes de PNHH. Si le patient est resistant au traitement, un adenome est recherche. Si cet adenome est diagnostique, il est enleve. S'il s'agit d'une forme diffuse, une pancreatectomie sub-totale est realisee avec une biopsie per-operatoire. En cas d'hyperplasie ou de forme mixte, nous recommandons une pancreatectomie totale et en cas de nesidioblastose, une pancreatectomie partielle.

Surgical acceptance of the laparoscopic splenectomy in the pediatric hematological disorders has improved due to safety, to relif postoperative pain, and to reduce the hospital stay. The aim of this study is present our surgical laparoscopic technique performed.Eight patients, 6 spherocytosis (age ranged: 6-14 years, average age: 9.5 years; 5 girls and 1 boy) and 2 cases of autoimmune hemolytic anemia (both boys of 18 months and 4 years) underwent laparoscopic splenectomy. The Trias-Targarona technique has been performed placing the patient at the right lateral-decubitus position. Four trocars are placed, two of 5 mm in the posterior axillary line and epigastrium and other 2 trocars of 12 mm, in the middle of them.In all these 8 patients a laparoscopic splenectomy was completely performed. We had 2 cases with problems with the sac introduction. Cosmetic results have been excellent and we changed the 7-day antibiotic protocol for 2-day protocol. The surgical time has changed from 3 to 2 hours.With laparoscopic splenectomy we can give an adequate response to hematological disorders in children with a acceptable surgical time, an excellent cosmetic result with a minimum postoperative time and a very short hospital stay.

Since Gross established the basic rules for nephrectomy in Wilms' tumour (WT) in 1953, the management of nephroblastoma has been more straightforward. However, some cases with intravascular involvement, currently detected by ultrasound (US), may represent a daunting challenge for the surgeon. Inferior vena cava with tumour thrombus, induced by WT, can be asymptomatic and, if undetected, can contribute to poorer prognosis for two main reasons: possible neoplastic cells inside the thrombus and higher morbidity risk of surgery. From 1979 to 1993, 81 WT were studied by routine US. Intracaval thrombosis was diagnosed in four (5%), in one of which the thrombus extended to the right atrium. In our experience, the surgical strategy in each of the four cases (100% survival) depended on the length of the thrombus and whether or not it infiltrated the vena cava wall. If the thrombus can be easily removed: complete resection. However, in cases of atrial thrombus, and more particularly if the thrombus involves the intima, we suggest the thrombus not be touched since the problem may be solved by preoperative and postoperative chemotherapy. Thus the favourable prognosis would be maintained and superfluous risky surgery avoided.

In 1987, we described the use of fibrin sealant for ensuring perfect haemostasis of the raw hepatic surface in reduced-size or partial paediatric liver transplantation. The success of our experimental and clinical studies led to new and wider surgical applications for this product. In liver transplantation, fibrin sealant was used to ensure high-pressure vascular anastomoses and to seal the raw surface in the reduced-sized liver. In these cases we sought efficacy in haemostasis, sealing and safety. The success of this high-risk surgery permitted us to employ fibrin sealant in other cases, including hepatic and splenic ruptures, complete tracheal stenosis treated with pericardial graft and thoracic and abdominal tumours, particularly bilateral Wilms’ tumours, in which extensive tumourectomy must be performed in both kidneys and where the risk of urine and blood leakage is high. For all these indications the aim was the same: sealing. We have had no side effects or morbidity due to fibrin sealant. Thus, it was later applied in two cases of lymphangioma to avoid recurrence. At present, in rat models we are studying the possible use of fibrin sealant to prevent impending perforation provoked by neonatal necrotizing enterocolitis (NEC) and have applied it successfully in one clinical case. In this particular case, we used fibrin sealant by spray to avoid perforation and fixed the intestinal segments to prevent occlusive adhesions, and we used fibrin sealant without spray to apply a layer between the peripheral small intestine and the abdominal zip that was placed to control NEC evolution daily. It seems clear that new applications for fibrin sealant are emerging and our experience provides wider possibilities in paediatric surgery.

The lack of availability of pediatric donors for liver transplant has resulted in a high mortality rate among children awaiting transplantation, especially in patients weighing less than 10 kg. Knowledge of techniques of liver reduction allows liver transplantation in low-weight children with an adult liver graft, although with increased risk. The authors studied 70 pediatric liver transplants, 14.3% of whom received a reduced-size organ. Maximal difference between donor and recipient weight was 7.7. Segments II, III and IV (right hepatectomy) were transplanted in eight cases while segments II and III were transplanted in two cases. Transfusional requirements during the 48 hour postoperative period were not significantly different between patients with reduced-size liver and patients who received the full-size organ. There was not morbi-mortality secondary to surgical technique in reduced liver transplant group of patients.

A 18 month-old boy with asplenic syndrome was admitted to our hospital with a twelve hours history of mucous vomiting, abdominal pain and a round epigastric mass. It was diagnosed as having a gastric volvulus and an emergency laparotomy was performed. The stomach was situated at the right side and a mesenterioaxial volvulus was found, with cyanosis of the antrum. The gastrophrenic and gastrosplenic ligaments were absent. The clinical and radiological characteristics of asplenic syndrome are reviewed. The pediatric surgeons must be aware of the digestive malformations of the asplenic syndrome, because some of that like malfixation of the stomach could be the cause of an acute abdomen.

Seventy-five percent to 80% of patients with biliary atresia (BA) will be candidates for paediatric liver transplantation (PLTx) throughout the first 14 years of life. They form the main group of recipients in our Paediatric Liver Transplant Unit. Of 48 children transplanted, 21 (44%) had BA. These patients present particular features, average weight of 12 kg, mean age of 3 years, and severe malnutrition prior to PLTx, which distinguish them from other paediatric candidates. With the advent of PLTx, portoenterostomy (PE) has ceased to be the only recourse for treating the majority of patients with BA. Different factors converge in these patients: some, including haemorrhage and adhesions, may present technical difficulties, and others, such as infections (19% in this series) due to severe malnutrition and malabsorption and consequent chronic rejection (14% in this series), often lead to death in the postoperative period (33% in this series). BA is treated by all paediatric surgeons, but the overall success rate now depends not only on PE but also on PLTx. In an attempt to facilitate possible later PLTx in pts with BA, the authors as paediatric surgeons performing PE or PLTx present surgical modifications and emphasize the most important medical aspects conducive to the improved general status of these pts. Our aim was to establish guidelines for taking full advantage of PE while preventing posterior problems and facilitating future transplant surgery.

The use of anorectal manometry as a biofeedback technique in the treatment of constipation and fecal incontinence in patients with spina bifida is described. The technique was applied in ten incontinent spina bifida patients aged 3 to 16 years. To evaluate our results we used the concept of “controlled incontinence,” i.e., voluntary defecation at set times, with the patient remaining “clean” the rest of the time as a base. A total of 80% satisfactory results was obtained.

Between 1966 and 1989, 165 cases of Hirschsprung's disease, of which 20 (12.1%) were total colonic aganglionosis, were surgically treated at our centre. In the first period up to 1978, in which total parenteral nutrition (TPN) was not used or was used with reservations, 6 of 11 patients died. From 1978 to 1989, 9 further cases were treated with the support of TPN and only 1 death occurred, in a child with anganglionosis up to one-half of the jejunum. The 13 surviving patients were treated with the Duhamel or Rehbein techniques; the Lester Martin procedure was not used. There were no notable complications. Follow-up of 10 of these patients, 2 of whom are now 20 years old, showed their general condition, height, weight, and food tolerance to be within normal limits. The number of daily bowel movements, on the average three, was higher than normal. Studies of serum iron transferrin, transferrin saturation, and vitamin B12 and folic acid absorption showed slightly low values that did not affect the erythrocyte count, hemoglobin, or hematocrit, but which require close monitoring. Hydrogen and fecal tests were normal.

To determine the metabolic response of newborns to surgical stress, 36 neonates between 2 h and 3 weeks of age and 11 infants of 1–3 months were included in a 7-year study. Plasma cortisol levels were determined by radioimmunoassay with blood samples taken before, during, and after surgery until they were within normal limits; the response to chemical stress (ACTH) was also studied and compared. The preliminary conclusions on cortisol response were: (1) absence of the circadian cycle in all neonates in the study; (2) a statistical relationship was observed between age and cortisol response; and (3) in newborns less than 9–10 days of age, the response to surgical stress was statistically different from that of newborns older than 10 days, whose response was similar to that of infants. These results suggest that there are two different stages during the neonatal period that correspond to different degrees of neuroendocrinological maturation.

It is difficult to find adequate donors for many children with low weight. In order to increase the pool size of donors and decrease mortality on the waiting list, several reduction techniques have been developed in the last years (reduced, segmental and split liver). The aim of this study is to compare morbility and mortality in our serie between children who received a full-size liver and those who received a partial one. We retrospectively compared 27 cases of reduced-size liver transplants (RLTx) with 102 cases of full-size liver transplants (FLTx) performed between june of 1985 and february of 2000. Mean age in RLTx was 38.1 months (range 6-144) vs 70.8 months (range 5-192) in FLTx. Mean weight was 11.9 k (range 5.8-30) in RLTx vs 20.48 k (range 4.4-68) in FLTx. Mean donor/recipient body ratio was 4.88 in RLTx and 2.03 in FLTx. The indication of transplantation was urgent in 14 patients (51.8%) from the RLTx group and in 12 (11.7%) from the FLTx one. The requirements of transfusions during surgery was greater in the RLTx (177 cc/kg of RBC transfusions vs 124 cc in FLTx). There was no differences between both groups regarding other postoperative complications (portal thrombosis, need of reintervention and biliary complications). Arterial thrombosis was observed only in FLTx (12 cases). Graft survival at 3 months was 49% for the RLTx and 73% for the FLTx. It was 43% and 67%, respectively, at 1 year, and 43% and 53% at 5 years after liver transplantation (p = 0.06). If we consider only elective transplants, survival was 72% for RLTx and 75% for the FLTx at 3 months. Although survival is lower in the RLTx group, the difference is not significant. If we consider only the elective transplants, survival is almost the same in both groups. The reduction techniques are a good method to decrease mortality in the waiting list without increasing post-transplant morbidity and mortality.

Orthotopic liver transplantation(OLT) is a therapeutic option for children with end-stage liver diseases. Once the OLT has been completed inmunosupression with prednisolone(P) and cyclosporine(C) is started. The two major problems in the postoperative period(POP)are related to rejection and drug induced hepatotoxicity (DIH) both manifested by similar byochemical and clinical symptomatology. To evaluate both problems is the aim of this paper. METHODS: The POP of 13 children that underwent OLT with succes, has been reviewed. The evidence of rejection or DIH, have been searched for. RESULTS: All patients presented in the POP a coagulopathy rise in serum transaminases, gamaGT, alkaline phosphatase and bilirrubin. 12 of them improved with P bolus, and later on, C levels and liver biopsy, proved to be a rejection crisis. Other one did not improve with P bolus, and subsequent C dosage and liver biopsy, confirmed DIH. CONCLUSIONS: If ther is doubt on rejection or DIH, P bolus must be administered in order to treat a possible rejection crisis, till the liver biopsy and other byochemical paramenters stablish the diagnosis with accuracy.