Background: Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly with a high rate of mortality and morbidity. Objective: Our aim was to determine a possible effect of standardized treatment on outcome in infants with CDH. Methods: All prenatally diagnosed patients with unilateral CDH born alive between January 2006 and December 2009 at the Erasmus MC or the University Hospital Mannheim were eligible for inclusion. Patients who underwent a fetal tracheal occlusion were excluded. From November 1, 2007, all CDH patients were treated according to a standardized treatment protocol. Patients were divided into two chronological groups according to their date of birth: without standardized treatment (group 1, Jan 2006–Oct 2007) and with standardized treatment (group 2, Nov 2007–Dec 2009). Outcome measures were mortality by day 28, bronchopulmonary dysplasia (BPD), defined as oxygen dependency at day 28, and need for extracorporeal membrane oxygenation (ECMO) therapy. Uni- and multivariate analyses were performed. Results: 167 patients were included. By day 28, 18% of the infants had died. Of the patients who were alive at day 28, 49% had BPD. An ECMO procedure was performed in 31% of the patients. Overall mortality for all included patients was 22%. In group 1, overall mortality was 33% and in group 2 overall mortality was 12% (p = 0.004). A standardized treatment protocol was independently associated with a reduced risk for mortality by day 28 (OR 0.28, 95% CI 0.11–0.68). Higher observed-to-expected lung-to-head ratios were independently associated with a lower risk for mortality by day 28 (OR 0.97, 95% CI 0.95–0.99), BPD (OR 0.97, 95% CI 0.94–0.98) and need for ECMO (OR 0.98, 95% CI 0.96–0.99). An intrathoracic position of the liver was independently associated with an increased risk for BPD (OR 3.12, 95% CI 1.41–6.90) and need for ECMO therapy (OR 3.25, 95% CI 1.54–6.88). Conclusion: Survival rates in patients with CDH increased significantly after the implementation of a standardized treatment protocol.

Five extremely low birth weight (ELBW) infants were treated in our institution for the milk curd syndrome, a milk bolus intestinal obstruction. The medical records of the five patients, who all underwent surgery, were retrospectively reviewed. History and examination are usually sufficient to diagnose the milk curd syndrome in ELBW infants. Confirmation with an abdominal X-ray may be possible but the X-ray findings may be mistaken for localised necrotising enterocolitis (NEC). Contrast enemas in an attempt to shift the bolus obstruction may be dangerous, and have a low success rate. Enterotomy and removal of the bolus is a safe and effective treatment if the diagnosis is early, otherwise resection with or without diverting ileostomy may be necessary. It appears that after years of disappearance, the milk curd syndrome has again become a clinical entity. It is probably due to a combination of high calcium and fat intake in an ever increasing population of ELBW infants.

Background: Cystic renal tumors are a diagnostic and therapeutic challenge. Cystic nephroma (CN), cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms’ tumor (CWT) are a spectrum with CN at the benign end, CWT at the malignant end and CPDN in the intermediate position. CN and stage 1 CPDN are often treated with surgery alone. International Society of Pediatric Oncology (SIOP) protocols for Wilms’ tumor (WT) advocate preoperative chemotherapy, which may be unnecessary and potentially harmful in CN and in stage 1 CPDN. There are difficulties in differentiating the three types using imaging techniques. Therefore, controversies exist regarding the optimal treatment. Methods: We describe 6 children, who each had a postoperative diagnosis of CN, CPDN or CWT, and we retrospectively evaluate the treatment strategies. Results: The three types cannot be differentiated using imaging techniques, although the presence of solid components in the tumor is indicative of WT. Conclusions: Surgery as first-line therapy should be seriously considered in children who have a cystic renal tumor, but further collaborative studies are needed since the distinction line between CPDN and CWT is not always clear.

BackgroundThoracoscopic surgery is an increasingly popular surgical technique to repair congenital diaphragmatic hernia (CDH). However, acidosis during surgery and the higher recurrence rate are considerable risk factors. The aim of this retrospective study is to compare the outcome of open versus thoracoscopic repair of the diaphragm in neonates with CDH with the same degree of cardiovascular and pulmonary illness who meet the criteria for thoracoscopic repair.MethodsRetrospective analysis of all patients of two large national reference centers for CDH born in the years 2008 through 2012, and meeting the criteria for surgical repair on cardiopulmonary and physiological criteria according to the CDH EURO consortium consensus and meeting the criteria for thoracoscopic repair according to the review by Vijfhuize et al. The surgical technical aspects were comparable in both centers.Results108 patients were included, of whom 75 underwent thoracoscopic repair and 34 underwent open repair. The gestational age and lung-to-head ratio were significantly lower and stay on the ICU significantly longer in the open-repair group. The operation time was longer (178 vs. 150 min, p = .012) and the recurrence rate higher (18.9 vs. 5.9 %, p = .036) in the thoracoscopic-repair group. The arterial pH, pO2, pCO2 and base excess before and after thoracoscopic repair were all significantly different.ConclusionAfter critical selection for thoracoscopic repair of left-sided CDH based on the patient’s preoperative condition, the outcomes of open repair were almost identical to those of thoracoscopic repair. A notable exception is the recurrence rate, which was significantly higher in the thoracoscopic-repair group. For the time being, thoracoscopic primary closure seems a safe and effective procedure, but efficacy of thoracoscopic patch repair has not been established.

In contrast to the data from the ELSO registry, we did not observe significantly more surgery-related bleeding complications after CDH repair on ECMO. Using a specific perioperative hemostatic treatment enabled us to perform CDH repair on ECMO with a low frequency of bleeding complications, thereby taking advantage of having the physiologic benefits of ECMO available perioperatively.

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Background/Objectives Central database registrations are widely used tools for assessment of clinical results, but their reliability is subject to debate. The aim of this study is to evaluate the reliability of central database registration for Wilms tumor (WT) nephrectomy‐related complications. Design/Methods All Dutch patients undergoing WT nephrectomy according to the International Society of Paediatric Oncology (SIOP) 2001 protocol between 2001 and 2013 were evaluated. Results from the central database were analyzed and compared with data found via individual medical records analysis (gold standard). Results A total of 179 patients were included. Fourteen (7.8%) patients with a total of 17 complications were identified in the central database. The medical records revealed that 33 (18.4%) of patients had undergone a total of 41 complications (P < 0.001). Operative complications were similar between the groups (P = 0.157). Eleven short‐term complications were noted in the central database versus 27 in the medical records (P = 0.059). Significantly more long‐term complications, namely, adhesive small‐bowel obstruction, were noted from the medical records compared with the central database (7 vs 1, respectively, P < 0.001). Postoperative chemotherapy was significantly delayed by on average 6 days (P < 0.0001) in patients with complications. No significant effect of complications on event‐free survival, overall survival, or the relapse rate was recorded. Conclusion Central database registrations underestimate the incidence of surgery‐related complications after WT nephrectomy and need to be regarded with caution.