Exploring cell death mechanisms in spheroid cultures using a novel application of the RIP3-caspase3-assay.

This study explores the application of the RIP3-caspase3-assay in heterogeneous spheroid cultures to analyze cell death pathways, emphasizing the nuanced roles of apoptosis and necroptosis. By employing directly conjugated monoclonal antibodies, we provide detailed insights into the complex mechanisms of cell death. Our findings demonstrate the assay's capability to differentiate between RIP1-independent apoptosis, necroptosis, and RIP1-dependent apoptosis, marking a significant advancement in organoid research. Additionally, we investigate the effects of TNFα on isolated intestinal epithelial cells, revealing a concentration-dependent response and an adaptive or threshold reaction to TNFα-induced stress. The results indicate a preference for RIP1-independent cell death pathways upon TNFα stimulation, with a notable increase in apoptosis and a secondary role of necroptosis. Our research underscores the importance of the RIP3-caspase3-assay in understanding cell death mechanisms in organoid cultures, offering valuable insights for disease modeling and the development of targeted therapies. The assay's adaptability and robustness in spheroid cultures enhances its potential as a tool in personalized medicine and translational research.

Retrospective Analysis of the Association of a Small Vestibular Aqueduct with Cochleovestibular Symptoms in a Large, Single-Center Cohort Undergoing CT.

BACKGROUND AND PURPOSE: Temporal bones in some patients with Ménière disease have demonstrated small vestibular aqueducts; however, the prevalence and clinical importance of small vestibular aqueducts remain unclear in patients without Ménière disease. This study correlates the presence of a small vestibular aqueduct with cochleovestibular symptoms. MATERIALS AND METHODS: Consecutive temporal bone CTs in adults from January to December 2020 were reviewed. The midpoint vestibular aqueduct size in the 45°-oblique Pöschl view was measured by 2 reviewers independently in 684 patients (1346 ears). Retrospective chart review for the clinical diagnosis of Ménière disease, the presence of cochleovestibular symptoms, and indications for CT was performed. RESULTS: Fifty-two of 684 patients (7.6% of patients, 62/1346 ears) had small vestibular aqueducts. Twelve patients (15/1346 ears) had Ménière disease. Five of 12 patients with Ménière disease (5 ears) had a small vestibular aqueduct. There was a significant correlation between a small vestibular aqueduct and Ménière disease (P < .001). There was no statistical difference between the small vestibular aqueduct cohort and the cohort with normal vestibular aqueducts (0.3-0.7 mm) regarding tinnitus (P = .06), hearing loss (P = .88), vertigo (P = .26), dizziness (P = .83), and aural fullness (P = .61). CONCLUSIONS: While patients with Ménière disease were proportionately more likely to have a small vestibular aqueduct than patients without Ménière disease, the small vestibular aqueduct was more frequently seen in patients without Ménière disease and had no correlation with hearing loss, vertigo, dizziness, or aural fullness. We suggest that the finding of a small vestibular aqueduct on CT could be reported by radiologists as a possible finding in Ménière disease, but it remains of uncertain, and potentially unlikely, clinical importance in the absence of symptoms of Ménière disease.

Prevalence of Hirschsprung-associated enterocolitis in patients with Hirschsprung disease.

PURPOSE: Hirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short-(S-HD), long (L-HD), TCA and the type of operation used. METHODS: A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis, segment length, and surgical procedure (Soave, Swenson, Duhamel) were included. Pooled prevalence and subgroup analysis have been calculated for pre-and postoperative HAEC. RESULTS: 4738 articles were identified from the literature search, among which 57 studies, including 9744 preoperative and 8568 postoperative patients, were included. The groups were sorted by length of the aganglionic segment for further analysis. The pooled prevalence for preoperative HAEC was 18.3% for all types, 15.2% for S-HD and 26.1% for TCA. The pooled prevalence for postoperative HAEC was in total 18.2% for all segment lengths and used techniques. Subgroup analysis showed no significant difference in the occurrence of postoperative enterocolitis between the three techniques. CONCLUSION: The prevalence of preoperative HAEC increases with segment length. However, pooled data suggest that the postoperative risk for developing HAEC, independently of the employed method and segment length, is comparable to the preoperative risk.

Retrospective Review of Midpoint Vestibular Aqueduct Size in the 45° Oblique (Pöschl) Plane and Correlation with Hearing Loss in Patients with Enlarged Vestibular Aqueduct.

BACKGROUND AND PURPOSE: Vestibular aqueduct measurements in the 45° oblique (Pöschl) plane provide a reliable depiction of the vestibular aqueduct; however, adoption among clinicians attempting to counsel patients has been limited due to the lack of correlation with audiologic measures. This study aimed to determine the correlation between midpoint vestibular aqueduct measurements in the Pöschl plane in patients with an enlarged vestibular aqueduct with repeat audiologic measures. MATERIALS AND METHODS: Two radiologists independently measured the midpoint vestibular aqueduct diameter in the Pöschl plane reformatted from CT images in 54 pediatric patients (77 ears; mean age at first audiogram, 5 years) with an enlarged vestibular aqueduct. Four hundred nineteen audiograms were reviewed, with a median of 6 audiograms per patient (range, 3-17; mean time between first and last audiograms, 97.4 months). The correlation between midpoint vestibular aqueduct size and repeat audiologic measures (pure tone average, speech-reception threshold, and word recognition score) using a linear mixed-effects model was determined. RESULTS: The mean midpoint vestibular aqueduct size was 1.78 mm (range, 0.81-3.46 mm). There was excellent interobserver reliability with intraclass correlation coefficients for the 2 readers measuring 0.92 (P < .001). Each millimeter increase in vestibular aqueduct size was associated with an increase of 10.5 dB (P = .006) in the pure tone average, an increase of 14.0 dB (P = .002) in the speech-reception threshold, and a decrease in the word recognition score by 10.5% (P = .05). CONCLUSIONS: Midpoint vestibular aqueduct measurements in the Pöschl plane are highly reproducible and demonstrate a significant correlation with audiologic data in this longitudinal study with repeat measures. These data may be helpful for clinicians who are counseling patients with an enlarged vestibular aqueduct using measurements obtained in the Pöschl plane.

Diagnostic Performance of Conebeam CT Pixel Values in Active Fenestral Otosclerosis.

BACKGROUND AND PURPOSE: Quantitative bone densitometry on multidetector CT of the temporal bone is a diagnostic adjunct for otosclerosis in its active (spongiotic) phase, but translating this technique to conebeam CT is limited by the technical variability of conebeam CT pixel values. The purpose of this study was to evaluate the performance of internally calibrated conebeam CT pixel value measurements that can enable the determination of active fenestral otosclerosis (otospongiosis). MATERIALS AND METHODS: This study included 37 ears in 22 patients with a clinical diagnosis of otospongiosis in those ears and 35 ears in 22 control patients without the diagnosis. Temporal bone conebeam CT was performed. ROIs were set anterior to the oval window, in the lateral semicircular canal bone island, and in a nearby aerated space. Mean conebeam CT pixel values in these regions determined the relative attenuation ratio of the area anterior to the oval window normalized to normal otic capsule bone and air. RESULTS: The relative attenuation ratio for cases of otospongiosis was significantly lower than that for controls (P < .001). Based on receiver operating characteristic analysis, the optimal cutoff relative attenuation ratio was 0.876, which had an accuracy of 97.2% for the diagnosis of otospongiosis. CONCLUSIONS: Internally calibrated pixel value ratios in temporal bone conebeam CT can feasibly help diagnose active/spongiotic-phase fenestral otosclerosis in an objective manner.

Intestinal CD8+ T cell responses are abundantly induced early in human development but show impaired cytotoxic effector capacities.

Gastrointestinal viral infections are a major global cause of disease and mortality in infants. Cytotoxic CD8+ T cells are critical to achieve viral control. However, studies investigating the development of CD8+ T cell immunity in human tissues early in life are lacking. Here, we investigated the maturation of the CD8+ T cell compartment in human fetal, infant and adult intestinal tissues. CD8+ T cells exhibiting a memory phenotype were already detected in fetal intestines and increased after birth. Infant intestines preferentially harbored effector CCR7-CD45RA-CD127-KLRG1+/- CD8+ T cells compared to tissue-resident memory CD69+CD103+CD8+ T cells detected in adults. Functional cytotoxic capacity, including cytokine and granzyme B production of infant intestinal effector CD8+ T cells was, however, markedly reduced compared to adult intestinal CD8+ T cells. This was in line with the high expression of the inhibitory molecule PD-1 by infant intestinal effector CD8+ T cells. Taken together, we demonstrate that intestinal CD8+ T cell responses are induced early in human development, however exhibit a reduced functionality. The impaired CD8+ T cell functionality early in life contributes to tolerance during foreign antigen exposure after birth, however functions as an immune correlate for the increased susceptibility to gastrointestinal viral infections in infancy.

Radioanatomic Characteristics of the Posteromedial Intraconal Space: Implications for Endoscopic Resection of Orbital Lesions.

BACKGROUND AND PURPOSE: Imaging is essential in the diagnostic work-up of patients with orbital lesions. The position of an orbital lesion relative to the inferomedial muscular trunk of the ophthalmic artery determines endoscopic resectability, anticipated technical difficulty, and patient morbidity. Although the inferomedial muscular trunk is not readily identifiable on preoperative imaging, we hypothesize that it is spatially approximate to the location where the ophthalmic artery crosses the optic nerve. Our aim was to determine whether the ophthalmic artery-optic nerve crosspoint anatomically approximates the inferomedial muscular trunk in a cadaver study and can be appreciated on imaging of known posteromedial orbital lesions. MATERIALS AND METHODS: Dissection was performed on 17 fresh-frozen cadaver orbits to assess the relationship between the inferomedial muscular trunk and ophthalmic artery-optic nerve crosspoint. Retrospective review of imaging in 9 patients with posteromedial orbital lesions assessed posteromedial orbital compartment characteristics and the ability to locate the ophthalmic artery-optic nerve crosspoint. RESULTS: In our cadaver study, the mean distance between the ophthalmic artery-optic nerve crosspoint and the inferomedial muscular trunk was 1.21 ± 0.64 mm. Retrospectively, the ophthalmic artery-optic nerve crosspoint was identifiable in 9/9 patients, whereas the inferomedial muscular trunk was not identifiable in any patient. Total or partial effacement of the posteromedial intraconal fat triangle was observed in 9/9 patients. CONCLUSIONS: This study of neurovascular relationships within the posteromedial orbit demonstrates that the ophthalmic artery-optic nerve crosspoint closely approximates the inferomedial muscular trunk and can be seen in patients with posteromedial orbital lesions. Posteromedial intraconal fat effacement may help to localize these lesions. These findings may facilitate multidisciplinary communication and help predict lesion resectability and patient outcomes.

Rescue Nuss procedure for inferior vena cava compression syndrome following posterior scoliosis surgery in Marfan syndrome.

PURPOSE: Scoliosis surgery in Marfan syndrome is common, even in the presence of a funnel chest. However, to date, no case has been reported with acute intra-/postoperative decompensation caused by vena cava compression following posterior spinal derotation and fusion. METHODS: A 15-year-old male patient with Marfan syndrome, a funnel chest and severe scoliosis was treated with surgery for the spinal deformity. Intraoperatively, the patient developed a clinically relevant compression of the inferior vena cava with severe circular depression. Postoperatively, a cava compression syndrome with severe pleural effusion, ascites and enormous swelling of the lower limbs was developed. A conservative treatment of the symptoms, consisting of thoracic drainage and negative fluid balance, failed. Subsequently, the patient was transferred to pediatric intensive care unit for further treatment. Echocardiography and a CT scan demonstrated cava compression syndrome. A rescue Nuss procedure of the funnel chest deformity was performed since conservative treatment failed. The clinical course proceeded without complications and with a decrease in clinical symptoms of inferior inflow congestion. The patient was discharged after almost 3 weeks. CONCLUSION: The problem of congenital stenosis of the inferior vena cava in Marfan syndrome has not yet been investigated. In the case of simultaneously existing funnel chest and scoliosis in Marfan syndrome, an interdisciplinary discussion is required to decide whether a repair of the funnel chest should be performed first in order to prevent a clinically relevant compression syndrome. For the detection of a preoperatively relevant stenosis of the inferior vena cava, an MRI or thoracic/abdominal CT should be used preoperatively.

Retrospective Review of Otic Capsule Contour and Thickness in Patients with Otosclerosis and Individuals with Normal Hearing on CT.

BACKGROUND AND PURPOSE: Otosclerosis is commonly identified on CT as a focus of hypodensity in the otic capsule anterior to the oval window. However, otosclerosis can have a sclerotic phase approximating the density of normal bone, making diagnosis challenging. This study assesses differences in otic capsule contour and thickness anterolateral to the anterior margin of the oval window in patients with otosclerosis compared with individuals with normal hearing. MATERIALS AND METHODS: Axial CT of 104 ears with clinically diagnosed otosclerosis and 108 consecutive ears of audiometrically normal individuals were retrospectively reviewed. Two radiologists independently evaluated the pattern of otosclerosis, otic capsule contour, and bone thickness on standardized axial images at the level of the oval window and cochleariform process. Measurements were made from the posterolateral margin of the cochlea to the apex of the otic capsule convex contour just anterolateral to the anterior margin of the oval window. In the absence of a convex contour, the sulcus between the oval window and the cochleariform process was identified, and measurement to the depth of the sulcus was used. Receiver operating characteristic analysis determined the best cutoff value of otic capsule thickness. RESULTS: Mean otic capsule thickness (2 SDs) was 3.08 (0.93) mm and 1.82 (0.31) mm in patients with otosclerosis and individuals with normal hearing, respectively (P < .001), with excellent interobserver agreement. Otic capsule thickness of >2.3 mm had 96.2% sensitivity, 100% specificity, 100% positive predictive value, and 96.4% negative predictive value for otosclerosis. A bulging/convex contour of the otic capsule had 68.3% sensitivity, 98.1% specificity, 97.3% positive predictive value, and 76.3% negative predictive value. CONCLUSIONS: Patients with otosclerosis have significantly thicker bone abutting the oval window than individuals with normal hearing.

Measurement for Detection of Incomplete Partition Type II Anomalies on MR Imaging.

BACKGROUND AND PURPOSE: Incomplete partition type II of the cochlea, commonly coexisting with an enlarged vestibular aqueduct, can be a challenging diagnosis on MR imaging due to the presence of a dysplastic spiral lamina-basilar membrane neural complex, which can resemble the normal interscalar septum. The purpose of this study was to determine a reproducible, quantitative cochlear measurement to assess incomplete partition type II anomalies in patients with enlarged vestibular aqueducts using normal-hearing ears as a control population. MATERIALS AND METHODS: Retrospective analysis of 27 patients with enlarged vestibular aqueducts (54 ears) and 28 patients (33 ears) with normal audiographic findings who underwent MR imaging was performed. Using reformatted images from a cisternographic 3D MR imaging produced in a plane parallel to the lateral semicircular canal, we measured the distance (distance X) between the osseous spiral lamina-basilar membrane complex of the upper basal turn and the first linear signal void anterior to the basilar membrane. RESULTS: The means of distance X in patients with normal hearing and prospectively diagnosed incomplete partition type II were, respectively, 0.93 ± 0.075 mm (range, 0.8-1.1 mm) and 1.55 ± 0.25 mm (range, 1-2.1 mm; P < .001). Using 3 SDs above the mean of patients with normal hearing (1.2 mm) as a cutoff for normal, we diagnosed 21/27 patients as having abnormal cochleas; 4/21 were diagnosed retrospectively. This finding indicated that almost 20% of patients were underdiagnosed. Interobserver agreement with 1.2 mm as a cutoff between normal and abnormal produced a κ score of 0.715 (good). CONCLUSIONS: Incomplete partition type II anomalies on MR imaging can be subtle. A reproducible distance X of ≥1.2 mm is considered abnormal and may help to prospectively diagnose incomplete partition type II anomalies.

[Therapy of paediatric pancreatic trauma]. / Therapie traumatischer Pankreasverletzungen im Kindesalter.

BACKGROUND: The goal of this clinical study is to evaluate diagnostics and therapeutical strategies for paediatric pancreatic trauma. It is assumed that conservative treatment is at least as good as operative treatment. PATIENTS / MATERIAL AND METHODS: In a retrospective study 36  children with pancreatic trauma treated between the years 1987 and 2010 in the paediatric surgery department of the University Medical Centre Mannheim were included. Injury grades, diagnostics and treatment were evaluated. RESULTS: In a collective of 36  patients with an average age of seven years the following injuries were found: 26  grade 1, three grade  2, five grade  3 and two grade  4 injuries. Eight patients presented with polytrauma, 20 showed additional injuries. Initial levels of amylase and lipase and screening ultrasound were not always sensitive. 18  patients received conservative treatment. The remaining 18  were operated: either exploratory laparotomy and drainage or pancreas reconstruction with inner stenting or distal pancreatic resection were performed. Pancreatic pseudocysts were drained by transgastric stenting. CONCLUSION: 20 years ago blunt pancreatic trauma was usually treated operatively. Today grade 1 and grade 2 injuries should be treated conservatively. Higher graded pancreatic ductal injuries can be managed operatively with ductal stenting and organ conservation. Pancreatic resections are not always necessary. Endoscopic transgastric drainage of persisting pancreatic pseudocysts is also successful in children.

A 20-year experience on neonatal extracorporeal membrane oxygenation in a referral center.

OBJECTIVE: Advances in treatment of neonatal respiratory failure are responsible for a decline in the number of newborns treated with extracorporeal membrane oxygenation (ECMO). The aim of this study are to determine demographic changes, focusing on time of referral, diagnosis, and respiratory parameters in neonates put on ECMO. DESIGN: Retrospective review. SETTING: Tertiary ECMO center. PATIENTS: A total of 321 neonates were treated with ECMO from January 1987 to December 2006. RESULTS: Overall number of patients increased with every 5-year period, whereby congenital diaphragmatic hernia (CDH) was the most common diagnosis (53%), followed by meconium aspiration syndrome (MAS) (21%), sepsis and/or pneumonia (13%), and others such as persistent pulmonary hypertension of the newborn (PPHN), respiratory distress syndrome (RDS), or hypoplasia of the lung (13%). Worsening severity of illness as measured by ECMO duration and days on ventilator has to be stated for all diagnoses. Nevertheless, survival rate remained stable; both overall and diagnosis-specific mortality rates did not change significantly. Of all children, 67% survived to discharge or transfer, while best rates were seen for MAS (94%), followed by sepsis and/or pneumonia (69%), CDH (62%), and other diagnoses (43%). Concerning survival rate, no difference between inborn and outborn children occurred. However, between early- and late-referred children, a referral to the ECMO center during the first 24 h of life was associated with a significantly higher rate of survival (77% versus 54%, p = 0.0004), predominantly seen for CDH (67% versus 35%, p = 0.02). CONCLUSION: We strongly recommend timely transfer to an ECMO center in patients with CDH who are at risk of circulatory failure.

The chronic liver disease in patients with short bowel syndrome: etiology and treatment.

Short bowel syndrome is a life threatening disease with a high mortality and morbidity. Since home parenteral nutrition (PN) has been established, there is an increasing number of patients surviving the acute loss of bowel function. But on the long-time these patients suffer from different complications of PN, with loss of central venous access, recurrent sepsis and finally the syndrome of progressive cholestatic liver disease. Both loss of central venous access and especially the progressive cholestatic liver disease are the limiting factor for the long-term survival of patients suffering from intestinal failure. Interestingly, the pathophysiologic mechanisms of PN induced intrahepatic cholestasis have not been dissolved yet and seem to be of multifactorial genesis. Cholestasis has shown to be associated with prematurity, recurrent sepsis, enteral and PN, especially with lipid emulsions. Enteral feeding and a well-controlled regime of PN lower the incidence of end-stage liver disease and, therefore, has to be optimized in the therapy of these patients.

Transanal pull-through procedure for Hirschsprung's disease: a 5-year experience.

BACKGROUND/PURPOSE: Transanal endorectal pull-through (TEPT) has become a widely used approach for the treatment of Hirschsprung's Disease. The technique is safe and, according to previous reports, it has a good clinical outcome. In this study our experience with TEPT in the early postoperative period is evaluated. METHODS: The clinical course of 34 children (28 boys and 6 girls) who underwent one-stage pull-through operation according to De la Torre for Hirschsprung's disease from January 2003 to December 2007 was reviewed. Their ages ranged from 2 months to 4 years. Complications occurring within the first four weeks after operation were analyzed. RESULTS: Eight of 34 children (24 %) had early complications in the form of dehiscences of the anastomosis. Two children (6 %) had symptomatic anastomotic dehiscences. One child had an almost full retraction of the colon that had to be pulled down and resutured. One child developed a retrorectal abscess three weeks postoperatively due to anastomotic leakage. The dehiscences of 6 children (18 %) were asymptomatic. These dehiscences were detected only with standardized routine examination. The dehiscences healed uneventfully after resuturing. Two other patients (6 %) developed an anastomotic stricture that could be treated with rectal dilatations. Four children (12 %) showed a single episode of postoperative enterocolitis. CONCLUSION: The rate of early clinical and particularly subclinical complications such as anastomotic dehiscences after TEPT is higher than previously estimated. Patients should be monitored carefully during the early postoperative period. Severe complications can only be avoided with a thorough examination. Early resuturing of dehiscences might be helpful to prevent hazardous sequelae.

[Evaluation of stents in treating childhood benign esophageal strictures]. / Evaluation von Stents zur Behandlung der benignen Osophagusstenose im Kindesalter.

BACKGROUND: Esophageal stenting is a popular of treatment of esophageal strictures in adults. It has also been described for children with benign strictures who did not respond to standard dilatation therapy. The aim of the study was to evaluate weather esophageal stents could be used safely and effectively in the treatment of benign esophageal strictures in children. PATIENTS: From 1993 to 2005 stenting therapy was performed in 12 children with complicated esophageal strictures. Etiologies of the strictures were caustic burns in 9 patients, postoperative strictures due to complicated esophageal atresia in 2 patients and iatrogenic esophageal injury in 1 patient. METHOD: Esophageal silicon tubi, covered retrievable expandable nitinol and plastic stents were placed endoscopically. The clinical course and the long term follow up were evaluated retrospectively RESULTS: The stents and tubi were placed in all patients without complications and were later removed successfully. 6 patients were treated with a self expanding plastic stent. The plastic stents showed a distinct tendency to migrate but in 5/6 patients esophageal stricture was treated successfully. 3 patients were treated by a covered self expanding nitinol stent. No migration occurred. One patient was asymptomatic after therapy, one required further dilatation therapy and the third had esophageal resection. 3 patients were treated by esophageal tubi. 2 patients required surgery in the follow up, one patient is asymptomatic. CONCLUSION: The use of stenting devices in children to treat benign esophageal strictures is safe and efficient. The self expanding plastic stents had the best long term results but required high compliance of parents and children due to the tendency of stent migration. Self expanding nitinol stents are more traumatic at the extraction procedure and are useful in patients with low compliance. Recurrence of strictures occurred most often after esophageal tubi possibly due to the lack of radial expansion.

Long-term outcome in patients with short bowel syndrome after longitudinal intestinal lengthening and tailoring.

OBJECTIVES: Longitudinal intestinal lengthening and tailoring (LILT) is a well-established surgical treatment for short bowel syndrome. It has been shown to enhance peristalsis, decrease bacterial overgrowth, and extend mucosal contact time for nutrients. We present the results of a long-term follow-up of patients who underwent LILT and define prognostic parameters for the survival of these patients. PATIENTS AND METHODS: Between 1987 and 2006, 53 patients underwent LILT in our institution. The main diagnoses were gastroschisis, intestinal volvulus, intestinal atresias, and necrotizing enterocolitis. LILT was performed at a mean age of 24 months (range 4144 months). The follow-up time was 79.76 months (range 6234 months). RESULTS: After LILT, 41 of 53 patients survived, and 36 of 41 surviving patients were successfully weaned from parenteral nutrition (PN). In long-term follow-up 79% stayed free of PN. The overall survival rate was 77.36%. Weight gain occurred in 58% of the patients after LILT. The quality of life after LILT is on a high level, with most patients having normal physical strength and participating in normal social life and education. Prognostic factors for survival after LILT in short bowel syndrome are length of small intestine (0.06582 + 0.0131 x bowel cm), length of large bowel (P = 0.039), preoperative liver function, and successful weaning from PN within 18 months postoperatively (P = 0.0032). CONCLUSIONS: Patients undergoing LILT in short bowel syndrome have a high survival rate, weight gain, and a high quality of life. Autologous gastrointestinal reconstruction remains therefore the first choice in the treatment of patients with short bowel syndrome.

The impact of longitudinal intestinal lengthening and tailoring on liver function in short bowel syndrome.

INTRODUCTION: Short bowel syndrome is a functional or anatomic loss of major parts of the small bowel leading to severe malnutrition. The limiting factor for the survival of these patients remains parenteral nutrition-related liver damage leading to end-stage liver failure. Longitudinal intestinal lengthening and tailoring (LILT) has been proven to enhance peristalsis, to decrease bacterial overgrowth and to extend the mucosal contact time for the absorption of nutrients. The aim of this study was to show the impact of LILT on the development of parenteral nutrition-related liver damage. PATIENTS AND METHODS: A cohort of 55 patients with short bowel syndrome managed with LILT in our institution between 1987 and 2007 was retrospectively reviewed. LILT was performed at a mean age of 24 months (range 4 - 150 months). Mean follow-up time was 83.76 months (range 5 - 240 months). We obtained reliable data from 31 patients with regard to liver enzymes and function parameters in blood samples before LILT and at the present time. Liver biopsy was performed in 14 patients prior to LILT. RESULTS: Liver enzymes ALAT (mean 121 U/l), ASAT (mean 166 U/l) and bilirubin (mean 2.49 mg/dl) were elevated preoperatively in 27/31 children. After the lengthening procedure, ALAT (mean 50 U/l), ASAT (mean 63 U/l) and bilirubin (mean 1.059 mg/dl) normalized except in 5 of 8 patients who could not be weaned from parenteral nutrition after LILT. Liver function parameters such as the international normal ratio (INR) were slightly elevated in 5/31 patients. Albumin was generally low, probably due to parenteral nutrition. Liver biopsy was performed in 14 patients preoperatively, showing 4 patients with low-grade, 6 patients with intermediate and 4 patients with high-grade fibrosis. End-stage liver disease with cirrhosis was an exclusion criterion for LILT. All patients with liver fibrosis showed a normalization of liver enzymes when they were weaned from parenteral nutrition. But patients with higher grade liver fibrosis tend to develop more complications perioperatively. CONCLUSION: After LILT, all patients with liver fibrosis who could be weaned from parenteral nutrition showed a normalization of liver enzymes. Preoperative liver biopsy is mandatory in order to differentiate reversible liver fibrosis from end-stage liver disease. A higher grade of liver fibrosis and elevated INR has been shown to be a sensitive parameter for peri- and postoperative complications.

[Treatment of lymphangiomas with picibanil in the first year of life]. / Behandlung von Lymphangiomen mit Picibanil im ersten Lebensjahr.

BACKGROUND: The therapeutic gold standard of cystic hygroma is its complete resection. Because of its growth pattern and its main location in the head and neck region complete resection is not always possible. An alternative is the local injection of Picibanil, but only few cases have been published about its use in infants. PATIENTS/METHOD: We retrospectively analyzed the data of 8 infants (age: 2 weeks-12 months) who got Picibanil therapy because of cystic hygroma in the time period 2002 until 2006. Follow up ranged from 3 months up to 3 years. RESULTS: During the postoperative period all patients had local swelling, in 4 cases accompanied with local inflammation and fever. Tumor reduction of >50% was obtained in 7 of 8 patients. CONCLUSIONS: Local injection of Picibanil in infants with cystic hygroma seems to be a safe alternative to surgical therapy, especially when complete tumor resection means damage of important neighbouring structures. Prospective trials are necessary to confirm the better outcome after therapy with Picibanil compare to primary surgery.

Efficacy of ERCP in infancy and childhood.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is a well established diagnostic and therapeutic tool in adult and pediatric patients. Because of its inherent risks we analysed our series in an attempt to better define the diagnostic and therapeutic roles of ERCP and its effectiveness in children. METHODS: All ERCPs performed in our department during a 10-year period in patients age 14 years and under were reviewed retrospectively. Indications, diagnostic findings, therapeutic interventions and final outcome were documented. RESULTS: A total of 61 procedures were performed on 47 patients whose median age was 8.56 years. ERCP was successful in 53 of 61 interventions (89%) with a complication rate of 6%. Thirty patients underwent diagnostic ERCP. In these a pathological diagnosis was established in 57%. In patients without morphological changes in ultrasound or magnet resonance imaging ERCP failed to set diagnosis too. Seventeen patients had therapeutic ERCPs. All of these interventions were performed with similar or better outcome than in adults. CONCLUSION: Indications for diagnostic ERCP are morphologic biliary or pancreatic duct disorders, but only where higher resolution imaging is necessary for therapeutic decisions. Without morphologic changes on ultrasound or magnetic resonance imaging we rarely see any indication for diagnostic ERCP. Efficacy of therapeutic ERCP in childhood is similar or even better to that in adults. Prognoses are directly dependent from the basic disease of the patient.