PURPOSE: The purpose of this study was to test the susceptibility of human hepatoblastoma and neuroblastoma cells to photodynamic diagnostics (PDD) and photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA) as a photosensitizer. METHODS: Cell cultures of human hepatoblastoma (HuH6) and neuroblastoma (MHH-NB-11) were incubated with 5-ALA at increasing concentrations to measure the cellular kinetics of photosensitization. After optimizing incubation parameters, the cell cultures were then irradiated with increasing light doses and cell viability was measured by CTB assay. Human fibroblastic cells served as controls. So far, only the hepatoblastoma cell line has been tested in vivo. After injection of HUH6 cells in immunoincompetent rats, the efficacy of PDT was assessed. Photosensitization was achieved by intraperitoneal injection of 5-ALA. The pharmacokinetics of different tissues was studied. In a second study, a PDT of implanted hepatoblastoma, liver and peritoneum was performed. The irradiated areas were excised 48 h after treatment and studied by microscopy. RESULTS: Cell culture experiments demonstrated a selective fluorescence for both tumor lines compared to controls. The photosensitized tumor cells demonstrated marked reductions in cell viability at significantly lower irradiation doses than the fibroblasts under PDT. The specificity of fluorescence was confirmed in vivo for hepatoblastoma, and all the sensitized and irradiated tumors showed marked phototoxic necrosis. CONCLUSION: Human hepatoblastoma and neuroblastoma demonstrate marked and specific fluorescence after the application of 5-ALA, making PDD possible. Cell death occurred in both cell lines after PDT in vitro. Additionally, hepatoblastoma was susceptible to PDT in an animal model. Further studies will be necessary to determine the role of PDT and PDD in a clinical setting.

Fetal malignant tumors are rare. We present a case of intrauterine diagnosis of a diaphragmatic tumor presenting with fetal hydrops at 32 weeks of gestation. The sonographic findings were bilateral pleural effusion, ascites and skin edema. A large right sided diaphragmatic tumor was identified. Due to ultrasound findings and magnetic resonance imaging (MRI) a solid malignant tumor was suspected. The pleural effusions were drained and malignant cells were identified. Due to rapid tumor progression a female hydropic newborn was delivered by caesarean section at 34 + 0 weeks of gestation. There was no sign of metastatic disease. Postnatally a tumor biopsy revealed an alveolar rhabdomyosarcom (RMS). The therapy included chemotherapy and secondary surgical intervention. After good primary response with complete remission after 6 month, the RMS relapsed at 12 months with cerebral metastasis. The prognosis is poor. This is the first case of fetal diaphragmatic RMS presenting with prenatal hydrops. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.

BACKGROUND Neural stem and progenitor cells of the Enteric Nervous System (ENS) are regarded as a novel cell source for applications in regenerative medicine. However, improvements to the current ENS cell culture protocols will be necessary to generate clinically useful cell numbers under defined culture conditions. Beneficial effects of physiologically low oxygen concentrations and/or the addition of anti-oxidants on propagation of various types of stem cells have previously been demonstrated. In this study, we tested the effects of such culture conditions on ENS stem and progenitor cell behavior. METHODS Enteric neural progenitor cells were isolated from postnatal day 3 mouse intestine and propagated either as monolayers or neurosphere-like bodies. The influence of hypoxic culture conditions and/or anti-oxidants on enteric cell propagation were studied systematically using proliferation, differentiation and apoptosis assays, whereas effects on gene expression were determined by qRT-PCR, western blot, and immunocytochemistry. KEY RESULTS Both hypoxic culture conditions and anti-oxidants supported a significantly improved enteric cell propagation and the generation of differentiated neural cell types. Enteric neural progenitors were shown to be specifically vulnerable to persistent oxidative stress. CONCLUSIONS & INFERENCES Our findings are consistent with previous reports of improved maintenance of brain stem cells cultured under reduced oxygen stress conditions and may therefore be applied to future cell culture protocols in ENS stem cell research.

Today liver transplantation is the only curative option for the treatment of end-stage liver diseases. A major limitation of liver transplantation is the donor organ shortage. Therefore, tissue engineering based cell transplantation is currently under investigation with the aim to replace liver tissue and function. The principle of tissue engineering is the notion of an interaction between a cell and a three-dimensional matrix. The matrix serves as a scaffold and guides a three-dimensional cell assembly. In addition, the matrix provides for a regulation of cell proliferation and function by cell-matrix interactions. In cultures of hepatocytes a regulation of cell proliferation and specific function by using three-dimensional matrices and by modifying the surface with isolated molecules of the extracellular matrix has been demonstrated. Furthermore, a beneficial effect of a flow bioreactor system on cell viability and function was observed. In addition, a system for heterotopic hepatocyte transplantation on polymeric matrices was developed in an animal model. In this transplantation model a long-term proliferation and function of transplanted hepatocytes was shown. The major limitation of matrix-based transplantation systems is the high initial cell loss, most probably due to an insufficient vascularisation. Thus, the development of vascularised matrices and the creation of bile ducts remain major problems in the technologies of hepatic tissue engineering and have to be addressed to enable further advances towards clinical applications.

Obesity in childhood and adolescents has gained epidemic proportions; in Germany 15-20 % of boys and girls are overweight, more than 6 % are known to be obese. By now, 25 % of relevant people show a pathological glucose intolerance, 4-5 % are developing type 2 diabetes mellitus (T2DM). In addition, metabolic disorders leading to hypertension and cardiac, renal or ophthalmological complications could be named as serious comorbidities. Medical and behavioural intervention as treatment for obesity in childhood remains largely ineffective: 5-10 % weight loss within 2 years rarely results in significant durable success. In adults, bariatric surgery is being used increasingly as an effective approach to achieve weight loss and to improve serious medical comorbidities, in particular T2DM. Enhancement of quality of life and explicit extension of survival are concomitant phenomenons. To date, a range of different types of bariatric procedures has been performed in adolescents, but studies evaluating and analysing preoperative data, postoperative course and follow-up in a representative number of patients younger than 18 years are still lacking. Nevertheless, current experience suggests significant weight loss and improving obesity-related medical comorbidities after bariatric surgery in adolescents too. Moreover, bariatric surgery in adolescents seems to induce less complications and a shorter hospital stay than in adults. Al-though surgical therapy for obesity in this group of patients remains an individual decision, even though explicit guidelines have been published specifying inclusion and exclusion criterias. Analysis of our own patient group and results of the study of the quality assurance "surgical treatment of morbid obesity" are appropriate tools to evaluate surgical techniques and to provide long-term follow-up.

Congenital malformations of the lung and diaphragm are a challenge in paediatric surgery. Depending on the malformation they show a broad spectrum of symptoms and a varying age at manifestation. Thus there are many diagnostic and therapeutic options, which require a good knowledge of the pathology. The treatment of these complex cases should lead to early referral to paediatric centres containing an interdisciplinary team with neonatologists, paediatric pulmonologists and cardiologists, ENT surgeons, anaesthesiologists, radiologists and paediatric surgeons. Some malformations are diagnosed prenatally and need intrauterine interventions. Decisive is the early diagnosis and treatment of these malformations. Nowadays the surgical therapy of neonates and infants with malformations of the lung and diaphragm is enriched by a number of endoscopic and endoluminal techniques, which are discussed critically in this article.

Congenital diaphragmatic hernia (CDH) occurs sporadically with an incidence of 1:2,500 live births. Despite the progress in neonatal intensive care, CDH remains associated with a mortality of at least 30 % in isolated cases. The in essence surgically correctable defect of the diaphragm enables the prenatal herniation of abdominal organs into the thoracic cavity. The resulting abnormal development of the airways and pulmonary vessels causes neonatal respiratory insufficiency and persistent pulmonary hypertension. The condition can be diagnosed prenatally and the degree of pulmonary hypoplasia, which determines the postnatal course, can be measured to make an -individual prognosis. In severely affected patients, prenatal surgery may improve neonatal outcome by reversing pulmonary hypoplasia. This is currently implemented by percutaneous fetoscopic endoluminal tracheal occlusion (FETO) to trigger fetal lung growth. Although there are no maternal complications, preterm rupture of the membranes remains the major drawback of the procedure (20 %< 34 weeks). However, as compared to historical controls of a similar severity, survival as well as early neonatal morbidity are significantly improved by FETO. As a consequence, a multicentre randomised-controlled trial in fetuses with moderate hypoplasia on FETO compared to expectant management has been started ( www.totaltrial.eu). Primary outcome measure is survival without chronic lung disease (i. e., with-out bronchopulmonary dysplasia). A trial in severely affected -fetuses with survival as main outcome is currently under review by ethics committee. A standardised neonatal management enables optimal treatment and multicentre compatibility. It remains to be proven if fetoscopic surgery can maintain a solid position in the prenatal treatment of CDH to improve both mortality and morbidity of the affected children.

The surgical management of craniopharyngiomas in children remains one of the more controversial topics in pediatric neurosurgery. Theoretically, the benign histology implies that total surgical excision would be sufficient to provide a cure. It has been widely established however, that in certain cases total excision may lead to unacceptable hypothalamic injury. The therapeutic goals for pediatric craniopharyngiomas therefore, require not just cure of the disease but also preservation of function. Over the last 15 years, there has been a growing worldwide advocacy for less extensive resection and for the utilization of multimodality therapy to limit morbidity. With this in mind, risk-adapted strategies designed to preserve hypothalamic structures have been developed. The preliminary results of these strategies appear to be encouraging. However, the long-term clinical outcome in terms of post irradiation complications and management of relapses is currently unknown.

Functional constipation is one of the most common gastrointestinal disorders. In both children and adults, most patients are managed conservatively with good results. In this review, we focus on the surgical approach to constipation. Patients who lack the capacity to consistently have voluntary bowel movements may need mechanical emptying of the colon through an enema program; for them, surgery to allow for antegrade enemas,(via the appendix or using a button device) is useful. Those patients with severe constipation not responsive to intense medical treatment may be candidates for other surgical interventions, such as resection of the dysfunctional colonic segment (rectosigmoid or whole colon), or plication,-pexy, and STARR techniques for evacuatory disorders secondary to obstructive anatomical features. Permanent stomas are an option of last resort.

BACKGROUND: The present study examines the causes and surgical management in children who underwent surgery for a mechanical Ileus. PATIENTS: We studied all children up to the age of 18 years who had undergone a surgical intervention for a mechanical ileus between 1.1.1996 and 31.12.2006. 89 children were included in this retrospective study. RESULTS: Of the total of 89 children 15 were newborn (16.9 %), 23 babies (25.8 %), 19 toddlers (21.3 %) and 32 school children (36 %) at the time of the operation. 51 of the 89 children had undergone at least one previous abdominal operation. Intraoperative findings showed the cause for the ileus to be adhesions in 56 and a bowel invagination in the remaining 11 children. Associated malformations were found in 34 children, the most frequent being malformations of the heart and gastrointestinal tract. The most frequent surgical intervention was adhesiolysis in 56 children (62.9 %), followed by the reposition of invaginated intestine in 11 (12.4 %). Bowel resection was necessary in 23 children (25.8 %). CONCLUSION: The risk for developing an ileus due to adhesions increases with the number of previous operations. Surgical intervention for an ileus aims to decompress the overstretched bowel and to restore gastrointestinal flow by removing the mechanical obstruction. One third of the children with an ileus have accompanying malformations. Children with a mechanical ileus should undergo surgery as soon as possible.

UNLABELLED Chronic constipation in children is common and produces significant morbidity. Identification of the site of dysmotility in constipation may determine the cause and permit directed management. Scintigraphy differentiates constipated patients with anorectal hold-up from those with colonic slowing. Adults with colonic slowing demonstrate variation in the site of hold-up. However, in children with colonic slowing, variability in the site of hold-up has not been investigated. PURPOSE The current study aimed to characterise colonic transit patterns in 64 children with chronic idiopathic constipation. METHODS Scintigraphic images were grouped visually by their transit patterns. Intra-observer variation was assessed. Scintigraphic data were analysed quantitatively. RESULTS Visual analysis of scintigraphy studies demonstrated normal transit (11/64), anorectal hold-up (7/64) and slow colonic transit (46/64). Transit characteristics in the slow transit group demonstrated three possible subgroups: pancolonic slowing (28/46), discrete hold-up in the transverse colon (10/46) and abnormal small and large bowel transit (8/46). Kappa testing demonstrated consistent characterisation (k = 0.79). Statistical analysis of scintigraphic data demonstrated highly significant differences from normal (P < 0.001) in the subgroups. CONCLUSION Scintigraphy demonstrates three possible transit patterns in children with chronic constipation secondary to slow colonic transit.

INTRODUCTION: Management of patients with chronic constipation (CC), irresponsive to medical treatment, is very difficult. There are some surgical approaches reported for the treatment. In this study we aimed to assess the results of different surgical procedures in patients with severe CC who were refractory to intensive medical treatments. METHODS: Fifteen patients with refractory chronic constipation underwent surgical management between 1998 and 2003 in Ankara University School of Medicine Department of General Surgery. RESULTS: Median age of the patients was 40 years (range, 24-77), female/male ratio was 11/4, median duration of symptoms was 13 years (range, 4-35 years) and median interval of two subsequent bowel movements was 15 (range, 5-30) days. Preoperative evaluation including barium enema, colonoscopy, colonic transit time, and cinedefecography and balloon expulsion test were done in all patients. Clinical analysis of constipation with these tests indicated a simple slow transit colon in three patients but more complicated variations of combined anatomical functional disorders in the rest of the cases. Surgical procedures consisted of total colectomy, Frykman-Goldberg procedure, Wells procedure and appendisostomy, laparoscopically in 8 of them. Deep vein thrombosis developed in the postoperative period after rectopexy and pelvic floor repair in one case. The median follow-up time was 5.5 years. Fourteen (93.3%) patients had an excellent bowel movement and were highly satisfied with the surgical management. CONCLUSION: Surgical interventions may be beneficial in selected patients with refractory chronic constipation (Tab. 2, Ref. 13). Full Text (Free, PDF) www.bmj.sk.

AIM: To assess the aetiology and the clinical patterns of chronic constipation in children. METHODS: A total of 78 patients (62% boys, mean age: 4.6 years) were enrolled in this retrospective study. For each patient, we collected these data: clinical features, radiological data, rectal manometry and rectal biopsy results, treatment and follow up. RESULTS: Functional constipation was the most frequent cause of chronic constipation (49 cases, 62.8%) followed by Hirschsprung's disease (19 cases, 24.3%). Rectal manometry, performed in all suspected Hirschsprung's disease, concluded to the absence of inhibitory rectoanal reflex in 17 of them. Rectal biopsy concluded to segmental absence of parasympathetic ganglion cells in eleven of them. Therapeutic approach consists of treatment of functional constipation by laxatives, enemas and dietary fibers in respectively 22, 12 and 6 children. Two other patients had a biofeedback re-education. Treatment was successful in 9 patients and unsuccessful in 2 others. Seven children with Hirschsprung's disease underwent Soave's (n = 3), Swenson's (n = 3) and Duhamel's procedures (n = 1). Outcome was favourable in five of them and complicated by stenosis in 2 others. CONCLUSION: Our study suggests that functional constipation is the most frequent cause of chronic constipation in children and that Hirschsprung's disease is the first organic cause of chronic constipation indicating the need of rectal manometry for diagnostic confirmation.

The authors report two cases of gastrointestinal bleeding in an emergency setting caused by angiodysplasia of colon and small bowel. They stress the rarity of the lesion and consider the difficulties involved in obtaining a preoperative diagnosis. The optimal management is uncertain and depends on the severity and rate of bleeding. A conservative medical approach is indicated for many patients, while surgery constitutes definitive treatment in case of massive hemorrhage or recurrent bleeding. In the cases reported the diagnosis was performed only via intra-operative enteroscopy. An unusual conservative surgical treatment was performed based on ligation of the vascular elements of the angiodysplasia. This method makes it possible to avoid an intestinal resection and yelds good results.

Less than 10% of patients with slow-transit constipation require surgical management after failure of medical treatment. Preoperative clinical, psychological and colorectal routine investigations (ie colonic transit test, anorectal manometry and defecography) are mandatory in order to highly select the patients. To day, the surgical management of slow-transit constipation consists of subtotal colectomy with ileorectal anastomosis, eventually by laparoscopic approach. Although, surgical management improves slow-transit constipation in two thirds of the patients, small bowel obstruction, abdominal pain and constipation recurrence can occur in 25%, 50%, and 10% of the patients respectively.

SUMMARY: The term intussusception refers to a spontaneous invagination of a portion of the intestine into another bowel loop. Its incidence is higher in children, but in adults it causes 1% to 5% of intestinal obstructions. The diagnosis of intussusception in the adult is difficult due to the variability of the symptoms. The condition may be chronic, intermittent, or acute. Surgical intervention is necessary in all cases and in up to 90% of cases an organic lesion inside the invaginated part of the bowel is found to be the lead point. The laparoscopic approach offers both a diagnostic and therapeutic option. Laparoscopy may be used as the final diagnostic or therapeutic tool for intussusception in the adult.

Chronic abdominal pain occurs in 17% of children aged 0-14 years with a peak of 33% at the age of 7 years. According to the Rome II criteria abdominal pain disorders can be classified as functional dyspepsia, irritable bowel syndrome, functional abdominal pain, abdominal migraine, and aerophagia. This new classification will hopefully lead to a more careful diagnosis of functional abdominal pain syndromes and to better treatment strategies. A thorough history taking and physical examination are the cornerstone of diagnostic workup in children with chronic abdominal pain. An extensive explanation and reassurance are the basis of an adequate treatment and in the majority of cases this is successful.