Stefan Holland-Cunz

Carbonic anhydrase IX correlates with survival and is a potential therapeutic target for neuroblastoma

Abstract Carbonic anhydrase IX (CAIX) is involved in pathological processes including tumorgenicity, metastases and poor survival in solid tumors. Twenty-two neuroblastoma samples of patients who were surgically treated at the University Medical Center Hamburg-Eppendorf were evaluated immunohistochemically for expression of CAIX. Results were correlated with clinical parameters and outcome. Neuroblastoma Kelly and SH-EP-Tet-21/N cells were examined for CAIX expression and inhibited with specific inhibitors, FC5-207A and FC8-325A. 32% of neuroblastoma tumors expressed CAIX. This was significantly associated with poorer survival. Kelly and SH-EP-Tet-21/N cells showed a major increase of CAIX RNA under hypoxic conditions. Proliferation of Kelly cells was significantly decreased by CAIX inhibitors, FC5-207A and FC8-325A, while proliferation of SH-EP-Tet-21/N cells was only significantly affected by FC8-325A. CAIX is a potent biomarker that predicts survival in neuroblastoma patients. CAIX-targeted therapy in neuroblastoma cell lines is highly effective and strengthens the potential of CAIX as a clinical therapeutic target in a selected patient collective.

Replaceable Jejunal Feeding Tubes in Severely Ill Children.

Long-term enteral nutrition in chronically ill, malnourished children represents a clinical challenge if adequate feeding via nasogastric or gastrostomy tubes fails. We evaluated the usefulness and complications of a new type of surgical jejunostomy that allows for easier positioning and replacement of the jejunal feeding tube in children. We surgically inserted replaceable jejunal feeding tubes (RJFT) connected to a guide thread which exited through a separate tiny opening of the abdominal wall. In a retrospective case series, we assessed the effectiveness and complications of this technique in severely ill children suffering from malnutrition and complex disorders. Three surgical complications occurred, and these were addressed by reoperation. Four children died from their severe chronic disorders within the study period. The RJFT permitted continuous enteral feeding and facilitated easy replacement of the tube. After the postoperative period, jejunal feeding by RJFT resulted in adequate weight gain. This feeding access represents an option for children in whom sufficient enteral nutrition by nasogastric tubes or gastrostomy proved impossible. Further studies are required to investigate the safety and effectiveness of this surgical technique in a larger case series.

Slow Recovery of Weight Bearing After Stabilization of Long-Bone Fractures Using Elastic Stable Intramedullary Nails in Children.

AbstractStabilization of diaphyseal long-bone fractures using elastic stable intramedullary nails (ESIN) in children promises early mobilization and rapid resumption of full weight bearing.We evaluated the duration of postoperative functional rehabilitation after ESIN, measured by the time from stabilization until first partial weight bearing, full weight bearing, and resumption of school sports.Fifty children with unstable, displaced fractures of the femur or lower leg treated with ESIN between 2002 and 2012 were included in this retrospective analysis. We classified fractures according to the pediatric comprehensive classification of fractures (PCCF).Thirty-five children sustained a femur fracture, and 15 children had a fracture of the lower leg or tibia. The surgeons in charge applied an additional plaster cast in 7 of 15 children who suffered a lower leg fracture. The postoperative time interval until full weight bearing in the group of children who had suffered transverse or short oblique femur fractures was significantly shorter (median: 4.4 weeks; range: 0.1–9.1 weeks) than that in the group who had sustained more complex fracture patterns (median: 6.8 weeks; range: 2.9–13.9 weeks; P = 0.04). Similarly, transverse and short oblique lower leg and tibia fractures required less time until full weight bearing (median: 4.1 weeks; range 2.7–6.0 weeks) than complex lower leg fractures (median: 6.1 weeks; range: 1.3–12.9 weeks; P = 0.04).ESIN proved fairly effective in restoring full weight bearing in transverse or short oblique fractures of the lower extremities but was less effective in complex fractures.

Laparoscopy-Assisted Single-Port Appendectomy in Children: Safe Alternative also for Perforated Appendicitis?

AbstractBecause of its low complication rate, favorable safety, cost-effectiveness, and technical ease, mono-instrumental, laparoscopy-assisted single-port appendectomy (SPA) has been the standard therapy for appendicitis in our department since its introduction 10 years ago. We report our experience with this technique and compare its outcome to open appendectomy (OA).The records of all children who underwent appendectomy at our institution over a period of 8 years were analyzed retrospectively. Patient baseline data, markers of inflammation, operative time, length of hospital stay, complication rate according to the classification of Clavien-Dindo, and histologic grading were assessed to compare the 2 surgical techniques (SPA and OA). The chi square test, the Students t test and the Wilcoxon-Mann-Whitney test were used to analyze the data and the comparisons of the mean values. A P value < 0.05 was considered significant.Overall, 975 patients were included in the study. A total of 555 children had undergone SPA and 420 had been treated by OA. Median operative time of SPA was longer than that of OA (60.8 min vs 57.4 min; P < 0.05). Length of hospital stay after SPA was shorter than after OA (4.4 days and 5.9 days, respectively; P < 0.001). The overall complication rate was lower for SPA than that for OA (4.0% vs 5.7%), but the difference of complications for SPA and OA was not statistically significant (P < 0.22). SPA was successfully performed in 85.9% of children. In 53.8% of patients with perforated appendicitis, no conversion was required. In the group of children with perforated appendicitis, the complication rate of ∼20% was independent of the surgical technique applied.With respect to operative time, length of hospital stay, and postoperative complication rate, SPA is not inferior to OA. SPA is safe and efficient, even in the management of perforated appendicitis.

Increased regulatory T cells in pediatric acute appendicitis

Appendectomy of an inflamed appendix has been reported to be protective in the pathogenesis of ulcerative colitis (UC) in both mice and human (1, 2). In mice, the contribution of a suppressive T cell subset has been described. However, no human prospective study has investigated the generation of those protective cells. Using immunofluorescence microscopy we evaluate for the first time the generation of suppressor T cells during pediatric acute appendicitis in a small control-matched cohort study. This article is protected by copyright. All rights reserved.

Unilateral Straight Hair—A Symptom of Acquired Horner's Syndrome in a Neonate

A multicystic tumor of the right neck was detected in a girl at 29 weeks of gestation by fetal ultrasound and magnetic resonance imaging (MRI). The baby was delivered by cesarean section at week 37 of gestation. The newborn adapted well, with minimal compromise of breathing and drinking. Postnatal ultrasound and MRI revealed a cervical lymphangioma measuring 60.5 × 60.6 × 41.2 mm. We performed subtotal resection of the tumor when the girl was 34 days. As a complication of surgical resection, the girl developed ipsilateral Horners syndrome. In the postoperative period, her curled hair turned straight at the side of the head affected by Horners syndrome. At the age of 2.5 years, ultrasonic imaging revealed the presence of three cysts measuring 3 mm in diameter each. Horners syndrome had improved, and the texture of the girls hair had become curly again on both sides.

Ovarian torsion in children: Development of a predictive score

Abstract The aim of this study was to identify clinical signs and symptoms of ovarian torsion (OT) in children and to develop a simple predictive score. A chart review of patients with acute adnexal pathologies treated at the University Childrens Hospital Basel, Switzerland, between March 2006 and June 2015 was performed. Medical records were screened for demographic and clinical data. These included clinical symptoms, laboratory studies, imaging, and type of treatment. The diagnosis OT was defined as intraoperative visualization of the torsed ovary around its pedicle at least 360 degrees. Variables predictive for OT were identified and the following score for the likelihood of having OT was developed: age (points = number of years) minus 3 points (if vomitus = “yes”) and plus 1 point (if “pain duration >12 hours”). A total of 80 patients with acute adnexal pathologies were identified. OT was recorded in 17 (21%) cases and ovarian cysts (OC) only in 63 (79%) cases. Patients who presented with OT were significantly younger than patients with OC only (P = .001). Correspondingly, 11 (65%) of the patients with OT had no menarche compared to 3 (5%) patients with OC only (P = .001). Vomiting (P = .001), a shorter pain duration (P = .01), and an elevated C-reactive protein (CRP) (P = .01) were observed significantly more often in patients with OT. The sensitivity of a positive OT score was 0.81 and increased to 1.00 if restricted to girls between 2 to 12 years of age. The presence of vomiting, short duration of abdominal pain, and elevated CRP level have a predictive value for the diagnosis of OT. In these patients, an exploratory laparoscopy should be conducted without delay. The presented OT score appears to be a helpful tool in diagnosing OT in children.

Single-center, retrospective study of the outcome of laparoscopic inguinal herniorrhaphy in children

Abstract Laparoscopic hernia repairs are used increasingly in children. The purpose of this single-center cohort observational research study was to analyze the outcome of children treated surgically for unilateral or bilateral inguinal hernia using laparoscopy. We did a STROBE-compliant retrospective outcome analysis of pediatric, laparoscopic hernia repair. Consecutive laparoscopic herniorrhaphies in 123 children done between March 2, 2010, and March 1, 2014, were included in this analysis. Data analysis was based on reviewing the hospital records and a prospective questionnaire. We evaluated postoperative hernia recurrence rate, occurrence of postoperative complications, duration of postoperative pain medication, and wound cosmesis. We first performed laparoscopic inguinal herniorrhaphy according to the techniques described by Schier et al and Becmeur et al in March 2010. We treated 46 girls and 77 boys with laparoscopically confirmed inguinal hernias, and their ages ranged from 0 to 16 years. Of these, 77 children suffered from unilateral hernias, 30 from unilateral hernias with contralateral patency of the vaginal process, and 16 from indirect bilateral hernias. The median follow-up interval was 38 months (range: 13–58 months). Overall, 8 (6.5%) of these 123 patients experienced a recurrence of the inguinal hernia. Two patients (1.6%) suffered a postoperative infection. Postoperative pain medication was administered by parents for 1 to 3 days in 67 (63.8%) of the 105 families who answered the question, and no pain medication was administered by 5 (4.0%) parents. Wound cosmesis was rated by the parents as invisible or barely visible in 106 (86.2%) of 123 patients and esthetically disturbing in 4 (3.2%) children. Laparoscopic inguinal hernia repair carries a learning curve and is safe and efficient in children thereafter. Further prospective studies are required to evaluate the long-term outcome of laparoscopic inguinal hernia repair in children.

Intradiaphragmatic hybrid lesion: surgical decision-making and value of minimal invasive surgery

Hybrid lesions (HLs) have elements of congenital pulmonary airway malformation and extrapulmonary sequestration (EPS) and belong to the congenital lung lesions. EPS usually arises in the thorax or the abdomen but rarely in the diaphragm. The preoperative diagnostic work-up based on chest radiograph, ultrasound (US) and CT often shows imprecise results. Therefore, the exact localisation of the lesion can only be ascertained intraoperatively. Here we present a patient, with an intradiaphragmatic HL, and demonstrate the difficulties of surgical decision making regarding the localisation of the lesion and discuss the value of minimal invasive surgery.