Sacral neuromodulation in children and adolescents with defecation disorders.

BACKGROUND: Even if understanding of neuronal enteropathies, such as Hirschsprung's disease and functional constipation, has been improved, specialized therapies are still missing. Sacral neuromodulation (SNM) has been established in the treatment of defecation disorders in adults. The aim of the study was to investigate effects of SNM in children and adolescents with refractory symptoms of chronic constipation. METHODS: A two-centered, prospective trial has been conducted between 2019 and 2022. SNM was applied continuously at individually set stimulation intensity. Evaluation of clinical outcomes was conducted at 3, 6, and 12 months after surgery based on the developed questionnaires and quality of life analysis (KINDLR). Primary outcome was assessed based on predefined variables of fecal incontinence and defecation frequency. KEY RESULTS: Fifteen patients enrolled in the study and underwent SNM (median age 8.0 years (range 4-17 years)): eight patients were diagnosed with Hirschsprung's disease (53%). Improvement of defecation frequency was seen in 8/15 participants (53%) and an improvement of fecal incontinence in 9/12 patients (75%). We observed stable outcome after 1 year of treatment. Surgical revision was necessary in one patient after electrode breakage. Urinary incontinence was observed as singular side effect of treatment in two patients (13%), which was manageable with the reduction of stimulation intensity. CONCLUSIONS: SNM shows promising clinical results in children and adolescents presenting with chronic constipation refractory to conservative therapy. Indications for patients with enteral neuropathies deserve further confirmation.

Centralization as the key survival benefit in acute neonatal surgery.

Introduction: Centralization of neonatal surgical care for congenital malformations is already under discussion. Acute care of neonatal emergencies in perinatal centers with affiliated hospitals is not uniformly regulated in Germany. Materials and methods: Analyses are based on acute pediatric surgical care at four affiliated hospitals of a perinatal center. Epidemiologic data and outcome parameters "survival", "intracerebral hemorrhage", and "revision of surgical indication" are assessed. Comparison is made between patients receiving surgical treatment at affiliated hospitals (group A) and patients with transfer to the university center for therapy in case of surgical indication for gastrointestinal diseases (group B). Results: 17 group A-patients are compared to 40 group B-patients. Comparison of epidemiological data reveals no significant differences. There is a survival advantage with transfer to the university center (mortality of 29% in group A vs. 2% in group B, p = 0.007). Intracerebral hemorrhage occurred more frequently in externally treated patients (group A 24% vs. group B 2%, p = 0.024). Surgical indication was revised in 30% of group B at the university center (p = 0.011) with consecutive successful conservative treatment. Conclusion: Transfer of patients at the beginning of the acute phase of gastrointestinal diseases is key to optimize the quality of neonatal surgical care. However, larger population studies should confirm the presented results, discuss restricting factors of real care structures and should rule out bias in triage of patients.

Sacral neuromodulation for constipation and fecal incontinence in children and adolescents - study protocol of a prospective, randomized trial on the application of invasive vs. non-invasive technique.

BACKGROUND: A therapeutic effect of sacral neuromodulation (SNM) on fecal incontinence (FI) and quality of life has been proven in adults. SNM is, however, rarely used in pediatric cases. The aim of the study is to investigate effects of SNM in pediatric constipation in a prospective parallel-group trial. METHODS: A monocentric, randomized, unblinded, parallel-group trial is conducted. SNM is conducted in the invasive variant and in an innovative, external approach with adhesive electrodes (enteral neuromodulation, ENM). We include patients with constipation according to the ROME IV criteria and refractory to conventional options. Patients with functional constipation and Hirschsprung's disease are able to participate. Participants are allocated in a 1:1 ratio to either SNM or ENM group. Clinical data and quality of life is evaluated in regular check-ups. Neuromodulation is applied continuously for 3 months (end point of the study) with follow-up-points at 6 and 12 months. Findings are analyzed statistically considering a 5% significance level (p ≤ 0.05). Outcome variables are defined as change in (1) episodes of abdominal pain, (2) episodes of FI, (3) defecation frequency, (4) stool consistency. Improvement of proprioception, influence on urinary incontinence, quality of life and safety of treatment are assessed as secondary outcome variables. We expect a relevant improvement in both study groups. DISCUSSION: This is the first trial, evaluating effects of neuromodulation for constipation in children and adolescents and comparing effects of the invasive and non-invasive application (SNM vs. ENM). TRIAL REGISTRATION: The study is registered with clinicaltrials.gov, Identifier NCT04713085 (date of registration 01/14/2021).

Differences in neonatal gastric tubes during insertion into a 3D model in relation to risk of potential perforation.

OBJECTIVES: Perforation of esophagus or stomach is a potential complication during and after insertion of a gastric tube in neonates. The aim of this study was to analyze different types of gastric tubes in a three-dimensional (3D) model of neonatal esophagus and stomach regarding potential perforations. METHODS: A 3D model of esophagus and stomach was created based on computed tomography data of a term neonate. Three types of gastric tubes were inserted into the 3D model, the localization was examined by radioscopy and the behavior, stiffness and manageability of each gastric tube was evaluated. RESULTS: Insertion of gastric tubes with higher stiffness was easier. The rates of correct localization differed significantly between the gastric tubes with the highest rate of correct localization in the softest tube (48.5%) and the lowest rate in the tube with the highest stiffness (21.2%). Additionally, the softest tube showed the lowest rate of localization of its tip at the stomach wall. CONCLUSIONS: The study illustrates differences between various types of gastric tubes regarding stiffness, behavior and resiliency. Softer gastric tubes may be beneficial. These differences may be relevant in neonatal care of very immature and very sick infants.

The Subdiaphragmatic Angle Of The Gastric Tube In Neonates - A Potential Predictor For Perforations?

BACKGROUND: Traumatic subdiaphramatic perforation of esophagus or stomach in neonates represents a rare clinical complication following the insertion of a gastric tube and is associated with high morbidity and mortality. The aim of the present study was to evaluate whether the angle of the gastric tube depicted on a plain radiograph may serve as a diagnostic tool to predict subdiaphragmatic traumatic (or iatrogenic) perforation. PATIENTS AND METHODS: Thoraco-abdominal radiographs of 128 preterm and term neonates without perforation and 15 neonates with perforation of the esophagogastric junction or the stomach were retrospectively analysed. RESULTS: The angle of the gastric tube increases with postnatal age (p=0.0380). In the control group, the angle of the gastric tube varied between 13° and 48° (median: 31°), whereas the values ranged from -31° to 42° (median: 11°) in neonates with subdiaphragmatic perforation. Perforation should thus be suspected in cases with an angle of<20° (sensitivity: 93.3%; specificity: 91.4%). Moreover, an unusual localization of the tip beyond the gastric bubble is highly indicative of perforation. DISCUSSION: The present study emphasizes the importance of verifying the correct course of an inserted gastric tube. CONCLUSIONS: In daily clinical practice, an angle of<20° should be followed by thorough physical examination, abdominal ultrasound and if applicable contrast medium examinations to exclude perforation.

A comparative observational study on intrasphincteric injections with Botulinum toxin vs. enteral neuromodulation in chronic refractory constipation.

PURPOSE: Botulinum toxin injections in the anal sphincter apparatus (Botox) and enteral neuromodulation (ENM) are options for treatment of refractory chronic constipation. We present a retrospective comparative observational study. PATIENTS AND METHODS: From 2014 to 2022, pediatric patients with chronic constipation were either treated with Botox or ENM with continuation of conservative treatment. Comparison was conducted regarding the primary outcome variables defecation frequency, stool consistency, and abdominal pain. Secondary outcomes were fecal incontinence, enuresis, change of medication and safety of treatment. RESULTS: 19 Botox patients (10 boys, 9 girls, 12 patients with Hirschsprung disease (HD), 7 patients with functional constipation (FC)) were compared to 24 ENM patients (18 boys, 6 girls, 12 HD patients, 7 FC patients). Groups differed significantly in age (5.0 years (Botulinum toxin) and 6.5 years (ENM), mean values, p-value 0.008). Improvement of constipation was seen in 68% (n = 13/19) of Botox and 88% (n = 21/24) of ENM patients (p = 0.153). Influence of etiology on therapeutic effects was not observed. Complications were minor. CONCLUSIONS: Botox and ENM can be considered as valuable and effective treatment options in refractory chronic constipation. Prospective, large-population studies should be designed to enable improved evidence.

Treatment of pediatric fistula-in-ano-Sphincter-sparing non-cutting seton placement as the future treatment of choice?

Background: Therapeutic principles of fistula-in-ano (FIA) are lacking evidence-based consensus on treatment options. Non-cutting, sphincter-sparing options have not been published for infancy and childhood FIA. Patients and methods: We are presenting retrospective data on FIA treatment with non-cutting seton placement between 2011 and 2020. Data were collected based on medical records and complemented by patients' contact for follow-up analyses between November 2021 and October 2022. Data were analyzed regarding the outcome variables of recurrent FIA and recurrent perianal abscess. Furthermore, outcomes in different age groups were compared (<1/1.5-12 years of age). Results: Treatment duration with non-cutting seton was at a median of 4.6 months and was not associated with recurrent FIA (p = 0.8893). Overall recurrence rate of FIA within an observation time of 9 months postsurgically was at 7% (n = 3/42) and was only seen in infancy, whereas recurrent perianal abscess was mainly observable in children (n = 2, p = 0.2132). Comparison of age groups revealed no significant differences. Of the 42 included patients, 37 responded in the follow-up analysis, resulting in a response rate of 88% with a median follow-up time of 4.9 years. Fecal incontinence was postsurgically only seen in two patients, who were diagnosed prior to surgery and symptoms remained unchanged. Conclusions: Non-cutting seton placement might be a promising option in the treatment of FIA in infancy and childhood. Perioperative settings like duration of placed seton and antibiotic treatment have to be discussed in further prospective, enlarged population-based studies.

Noninvasive Sacral Neuromodulation in Children and Adolescents: A Case-Control Study of Patients With Chronic Refractory Constipation.

OBJECTIVES: In adult patients with chronic refractory constipation, invasive sacral neuromodulation (SNM) has been applied successfully. There is a need for less invasive solutions while providing comparable therapeutic effects in children and adolescents. We present a prospective, interventional case-control study on the application of noninvasive SNM. MATERIALS AND METHODS: Patients with chronic constipation refractory to conservative treatment were prospectively included in the study from 2018 to 2021 and randomized to either SNM (SNM group: single current stimulation for 24 h/d, frequency 15 Hz, pulse width 210 µs, intensity 1-10 mA) or conventional treatment (controls: full range of pharmacologic and nonpharmacologic options). Treatment was conducted for 12 weeks. Treatment effects were collected with specialized questionnaires and quality-of-life analysis (KINDLR). Outcome variables were defecation frequency, stool consistency, fecal incontinence (FI) episodes, and abdominal pain. RESULTS: Analysis was conducted in 28 patients with SNM and 31 controls (median age 7.0, range 3-16 years). Overall responsiveness to treatment was 86% of the SNM group and 39% of the control group (p < 0.001). All outcome variables were positively influenced by SNM treatment. Defecation frequency improved in 46% of patients with SNM and in 19% of controls (p = 0.026), as did stool consistency in 57% of patients with SNM and in 26% of controls (p = 0.014). Fecal incontinence was significantly reduced in 76% of patients with SNM (n = 16/21 vs 42% of controls [n = 11/26], p = 0.042). Quality of life improved significantly during SNM treatment (71.32 [baseline] vs 85.00 [after 12 weeks], p < 0.001) and confirmed a positive influence of SNM treatment compared with the control group (85.00 [SNM after 12 weeks] vs 79.29 [controls after 12 weeks], p = 0.047). CONCLUSIONS: Outcome of noninvasive SNM treatment in patients with chronic refractory constipation is better than conventional treatment.

Prediction of High Bell Stages of Necrotizing Enterocolitis Using a Mathematic Formula for Risk Determination.

Necrotizing enterocolitis (NEC) continues to cause high morbidity and mortality. Identifying early predictors for severe NEC is essential to improve therapy and optimize timing for surgical intervention. We present a retrospective study of patients with NEC, treated between 2010 and 2020, trying to identify factors influencing the severity of NEC. Within the study period, 88 affected infants with NEC or NEC-like symptoms are analyzed. A multiple logistic regression analysis reveals the following three independent predictors for NEC in Bell stage III: red blood cell transfusion (p = 0.027 with odds ratio (OR) = 3.298), sonographic findings (p = 0.037; OR = 6.496 for patients with positive vs. patients without pathological findings) and cardiac anatomy (p = 0.015; OR = 1.922 for patients with patent ductus arteriosus (PDA) vs. patients with congenital heart disease (CHD); OR = 5.478/OR = 2.850 for patients with CHD/PDA vs. patients without cardiac disease). Results are summarized in a clinical score for daily application in clinical routine. The score is easy to apply and combines clinically established parameters, helping to determine the timing of surgical intervention.

Increased expression of OLFM4 and lysozyme during necrotizing enterocolitis in neonates: an observational research study.

BACKGROUND: In neonatal patients with necrotizing enterocolitis (NEC) the inflammatory response is mediated by a plurality of different proteins. The proteins olfactomedin 4 (OLFM4) and lysozyme (LYZ) are part of the intestinal mucosal defense and especially OLFM4 has rarely been evaluated in neonatal gastrointestinal diseases. The aim of this study was to analyze whether expression levels of both proteins of innate immunity, OLFM4 and lysozyme, were increased during NEC in neonates. METHODS: Intestinal tissues of patients with NEC were examined with immunohistochemical staining of formalin-fixed and paraffin-embedded sections of resected tissue using antibodies against OLFM4 and lysozyme. Staining-positive tissues were semi-quantitatively scored from 0 (no staining), 1 (weak staining), 2 (moderate staining) to 3 (highly intense staining) by two individual investigators. Intestinal tissue of infants with volvulus was used as a control as other intestinal tissue without major inflammation was not available. RESULTS: Both applied antibodies against OLFM4 showed different staining patterns with higher staining intensity of the antibody OLFM4 (D1E4M). OLFM4 (median score of the antibody OLFM4 (D1E4M): 3.0) and lysozyme (median score: 3.0) are highly expressed in intestinal and immune cells during NEC. Expression of OLFM4 and lysozyme in the control samples with volvulus was observable but significantly lower (median score of the antibody OLFM4 (D1E4M): 1.25; median score of the antibody against LYZ: 2.0; p = 0.033 and p = 0.037, respectively). CONCLUSIONS: Both proteins, OLFM4 and lysozyme, may play a role in the pathogenesis of NEC in neonatal patients, but the exact mechanisms of OLFM4 and lysozyme function and their role in immunological responses have not yet been resolved in detail. These observations add new insights as basis for further large-scale population research.

DMBT1 expression and neutrophil-to-lymphocyte ratio during necrotizing enterocolitis are influenced by impaired perfusion due to cardiac anomalies.

BACKGROUND: Deleted in malignant brain tumors 1 (DMBT1) is involved in innate immunity and epithelial differentiation. It has been proven to play a role in various states of inflammation or hypoxia of fetal gastrointestinal and pulmonary diseases. Discrimination of pathogenesis in necrotizing enterocolitis (NEC) based on cardiac status improves the understanding of NEC in different patient subgroups. We aimed at examining DMBT1 expressions regarding their association with cardiac status leading to impaired intestinal perfusion, intraoperative bacteria proof, and a fulminant course of NEC. METHODS: Twenty-eight patients with NEC were treated surgically between 2010 and 2019 at our institution. DMBT1 expression was examined in intestinal sections using immunohistochemistry to detect DMBT1 protein. Associations of clinical parameters and DMBT1 expression were analyzed. RESULTS: We examined DMBT1 levels in 10 patients without cardiac defects and 18 patients with persisting ductus arteriosus (PDA) and congenital heart defects (CHD). Compared to patients without cardiac malformations, DMBT1 levels tended to score higher in patients with PDA/CHD (p = 0.2113) and were negatively correlated with C-reactive protein in these infants (p = 0.0172; r = - 0.5533). The number of DMBT1-expressing macrophages was elevated in the PDA/CHD-subgroup (p = 0.0399). Ratios of neutrophils and monocytes to lymphocytes were significantly higher in infants with PDA/CHD (p = 0.0319 and 0.0493). DMBT1 expression was significantly associated with positive bacterial culture of intraoperative swabs (p = 0.0252) and DMBT1 expression of the serosa was associated with a fulminant course of NEC (p = 0.0239). CONCLUSIONS: This study demonstrates that DMBT1 expression may be influenced by cardiac anomalies with an impaired intestinal perfusion in the neonatal intestine. NEC in PDA/CHD infants is associated with more DMBT1-positive macrophages and a significantly elevated neutrophil-to-lymphocyte ratio.

Rare Tumors in Children and Adolescents - the STEP Working Group's Evolution to a Prospective Registry.

Background Very rare tumors (VRT) in children and adolescents have such a low incidence that until recently, they have not been integrated into the clinical and scientific network of pediatric oncology. Data is very limited and consistent treatment strategies are missing. Thus, VRTs are classic orphan diseases. To counteract this problem, the Arbeitsgemeinschaft für Seltene Tumorerkrankungen in der Pädiatrie (STEP) was founded. Here we report on patient recruitment during the first 10 years. Patients Patients aged up to 18 years and not included in any other clinical trial or GPOH registry were included in this analysis. Methods Data was collected from 2008 to 2018 by means of a standardized form. The recorded diagnoses were descriptively analyzed focusing on histology, localization, and year of report. Results A total of 623 patients with VRTs were registered. During 2008-2014, the annual number of registrations was around 40 and is around 90 since 2015. Most frequent diagnoses included tumors of the skin (n=150), tumors of the gastrointestinal tract (n=102), tumors of the gonads (n=77), the ENT region (n=68), and miscellaneous tumors (n=107). Discussion With the establishment of central structures for clinical consultation and documentation of VRTs, the number of registrations increased. Comprehensively, VRTs are as common as other classic pediatric oncology tumors, but extremely heterogeneous in terms of localization, histology, and prognosis. By a centralized and complete registration and analysis of VRTs, also in collaboration with international partners, it is possible to develop treatment strategies and thus greatly increase treatment quality.

Cholelithiasis and cholecystitis in children and adolescents: Does this increasing diagnosis require a common guideline for pediatricians and pediatric surgeons?

BACKGROUND: In contrast to adults, for whom guidelines on the cholelithiasis treatment exist, there is no consistent treatment of pediatric patients with cholelithiasis throughout national and international departments, most probably due to the lack of evidence-based studies. METHODS: We evaluated the German management of pediatric cholelithiasis in a dual approach. Firstly, a retrospective, inter-divisional study was established, comparing diagnostics and therapy of patients of the pediatric surgery department with the management of patients aged < 25 years of the visceral surgery department in our institution over the past ten years. Secondarily, a nation-wide online survey was implemented through the German Society of Pediatric Surgery. RESULTS: Management of pediatric patients with cholelithiasis was primarily performed by pediatricians in the retrospective analysis (p < 0.001). Pediatric complicated cholelithiasis was not managed acutely in the majority of cases with a median time between diagnosis and surgery of 22 days (range 4 days-8 months vs. 3 days in visceral surgery subgroup (range 0 days-10 months), p = 0.003). However, the outcome remained comparable. The hospital's own results triggered a nation-wide survey with a response rate of 38%. Primary pediatric medical management of patients was confirmed by 36 respondents (71%). In case of acute cholecystitis, 22% of participants perform a cholecystectomy within 24 h after diagnosis. Open questions revealed that complicated cholelithiasis is managed individually. CONCLUSIONS: The management of pediatric cholelithiasis differs between various hospitals and between pediatricians and pediatric surgeons. Evidence-based large-scale population studies as well as a common guideline may represent very important tools for treating this increasing diagnosis.

Association of clinical factors with postoperative complications of esophageal atresia.

BACKGROUND: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) remains one of the most common gastrointestinal neonatal malformations. Even though postoperative management is standardized, it differs between hospitals and disease-associated clinical factors that may play a role in outcome have not yet been assessed in detail. METHODS: In this monocentric retrospective study, data of 43 patients with EA between 2010 and 2018 were analyzed. Analysis includes assessment of the clinical background, surgical technique, postoperative management including application of continuous muscle relaxation (CMR), influence of coagulation parameters such as factor XIII and incidence of complications. RESULTS: 21 patients (49%) were preterm infants with birth weights between 490 and 2840 g (median 1893 g). Only 35% (n = 15) presented without any concomitant malformations. Within the entire study population, representing Vogt II, IIIb and IIIc, we observed an association between the development of a postoperative pneumothorax and anastomotic failure (AF) (p = 0.0013). Furthermore, pneumothorax was associated with anastomotic stenosis (AS) in Vogt IIIb patients (p = 0.0129). CMR (applied since March 2017 in 7 patients in an attempt to prevent anastomotic problems due to high complication rates) and coagulation factor XIII did not significantly correlate with postoperative outcome. CONCLUSION: Appearance of pneumothorax was correlated with postoperative complications. These children should be monitored carefully in closer scheduled gastroenterological follow-up esophago-gastro-duodenoscopies. CMR and factor XIII substitution did not reduce anastomotic leakage but should be tested within an enlarged study population.

Clinical Characteristics of Necrotizing Enterocolitis in Preterm Patients With and Without Persistent Ductus Arteriosus and in Patients With Congenital Heart Disease.

Background: Diagnosis and management of NEC is based on clinical, radiological, and laboratory findings. Discrimination of pathogens for an improved understanding of NEC in preterm infants and NEC in infants with congenital heart disease has been previously discussed and enables evaluation of further NEC biomarkers. Patients and Methods: Within a study period of 11 years (2008-2019), we identified 107 patients with a diagnosis of NEC at our primary care center. Thirty-six out of 54 patients suffering from NEC in high Bell stages who underwent surgery met inclusion criteria. These patients were classified according to their cardiac status, and analyses of clinical factors influencing NEC were conducted. Additionally, clinical factors associated with a fulminant course of NEC were examined. Univariable and multivariable analyses were performed. Results: The study populations consisted of 12 preterm infants with NEC but without patent ductus arteriosus (PT-NEC), seven preterm infants with NEC and patent ductus arteriosus (PDA-NEC), and 17 infants with NEC and congenital heart disease (CHD-NEC). Blood flow in intestinal vessels was impaired in infants with PDA-NEC and CDH-NEC. Therefore, we used logistic regression to compare PDA-NEC and CDH-NEC infants with PT-NEC infants: positive bacterial culture of intraoperative swabs (p = 0.0199; odds ratio: 21.9) and macroscopic intestinal necrosis (p = 0.0033; odds ratio: 43.5) were observed more frequently in the first group. Furthermore, multiple regression analysis determined the NEC localization (p = 0.0243) as a significant factor correlated with a fulminant course. Compared to a NEC exclusively localized in the colon, there is a 5.8-fold increased risk of a fulminant course when the small intestine is affected and a 42-fold increase of risk when both small intestine and colon were affected. Conclusion: An early diagnosis and timely surgical intervention of NEC, especially in infants with PDA and CDH may be considered to avoid major bowel necrosis (resulting in loss of intestinal tissue) and multiple operations.

A Pilot Study of Non-invasive Sacral Nerve Stimulation in Treatment of Constipation in Childhood and Adolescence.

Background/Aims: Constipation shows both, a high prevalence and a significant impact. However, it is often perceived as minor and treatment choices are limited. The neuromodulation approach is a valuable option to be considered. This study assesses the use of non-invasive sacral nerve stimulation to reduce constipation in children. Methods: Between February 2013 and May 2015, pediatric patients with chronic constipation were treated with this non-invasive neuromodulation procedure, adapted from classical sacral nerve stimulation. A stimulation device attached to adhesive electrodes on the lower abdomen and back generated an electrical field with a stable frequency of 15 Hz via variable stimulation intensity (1-10 V). The effect of therapy was evaluated in routine check-ups and by specialized questionnaires. Results: The study assessed non-invasive sacral nerve stimulation in 17 patients (9 boys, 8 girls, mean age 6.5 years). They underwent stimulation with 6-9 V for a mean of 11 h per day (range 0.5-24 h) over a mean of 12.7 weeks. Improvement of constipation was achieved in more than half of the patients (12/17) and sustained in almost half of these patients (5/12). Complications were minor (skin irritation, electrode dislocation). Conclusions: Non-invasive sacral nerve stimulation appears to be effective in achieving improvement in pediatric patients with chronic constipation. As an additional external neuromodulation concept, this stimulation may represent a relevant addition to currently available therapeutic options. Further studies are needed to confirm these results.

Pediatric Cullen gangrene: Case report of postoperative pyoderma gangrenosum in a preterm infant with a complex gastrointestinal malformation.

INTRODUCTION: Pyoderma gangrenosum (PG) is a rare condition in infants, children and adolescents. The postoperative variant of this rapidly progressive ulceration is known as Cullen gangrene. Only a few case reports of pediatric Cullen gangrene have been published. PRESENTATION OF CASE: We present a male infant (gestational age 31 + 6 weeks) with gastroschisis and intrauterine volvulus. After multiple operations due to his complex gastrointestinal malformation, the patient showed classical symptoms of postoperative PG (PPG) 4 weeks after birth. Diagnosis could be made early in an interdisciplinary approach, differentiating intraabdominal infection (vancomycin resistant enterococcus (VRE), candida albicans) from epifascial occurrence. Treatment with high-dose steroids and immunoglobulins was initiated immediately and led to rapid recovery. DISCUSSION: The unusual combination of a complex gastrointestinal malformation and an intraabdominal infection with VRE and candida albicans made the diagnosis difficult. However, early diagnosis is crucial in the treatment of Cullen gangrene. CONCLUSION: This is the first case report of Cullen gangrene presenting in an infant (at 4 weeks of age).