Awareness and diagnosis for intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS) in neonatal (NICU) and pediatric intensive care units (PICU) - a follow-up multicenter survey.

BACKGROUND: Constantly elevated intra-abdominal pressure (IAH) can lead to abdominal compartment syndrome (ACS), which is associated with organ dysfunction and even multiorgan failure. Our 2010 survey revealed an inconsistent acceptance of definitions and guidelines among pediatric intensivists regarding the diagnosis and treatment of IAH and ACS in Germany. This is the first survey to assess the impact of the updated guidelines on neonatal/pediatric intensive care units (NICU/PICU) in German-speaking countries after WSACS published those in 2013. METHODS: We conducted a follow-up survey and sent 473 questionnaires to all 328 German-speaking pediatric hospitals. We compared our findings regarding awareness, diagnostics and therapy of IAH and ACS with the results of our 2010 survey. RESULTS: The response rate was 48% (n = 156). The majority of respondents was from Germany (86%) and working in PICUs with mostly neonatal patients (53%). The number of participants who stated that IAH and ACS play a role in their clinical practice rose from 44% in 2010 to 56% in 2016. Similar to the 2010 investigations, only a few neonatal/pediatric intensivists knew the correct WSACS definition of an IAH (4% vs 6%). Different from the previous study, the number of participants who correctly defined an ACS increased from 18 to 58% (p < 0,001). The number of respondents measuring intra-abdominal pressure (IAP) increased from 20 to 43% (p < 0,001). Decompressive laparotomies (DLs) were performed more frequently than in 2010 (36% vs. 19%, p < 0,001), and the reported survival rate was higher when a DL was used (85% ± 17% vs. 40 ± 34%). CONCLUSIONS: Our follow-up survey of neonatal/pediatric intensivists showed an improvement in the awareness and knowledge of valid definitions of ACS. Moreover, there has been an increase in the number of physicians measuring IAP in patients. However, a significant number has still never diagnosed IAH/ACS, and more than half of the respondents have never measured IAP. This reinforces the suspicion that IAH and ACS are only slowly coming into the focus of neonatal/pediatric intensivists in German-speaking pediatric hospitals. The goal should be to raise awareness of IAH and ACS through education and training and to establish diagnostic algorithms, especially for pediatric patients. The increased survival rate after conducting a prompt DL consolidates the impression that the probability of survival can be increased by timely surgical decompression in the case of full-blown ACS.

Neonatal aortic arch thrombosis: analysis of thrombophilic risk factors and prognosis.

UNLABELLED: Arterial thrombosis in neonates and children is a rare event and is often associated with external risk factors such as asphyxia or sepsis. We report our experiences with two neonates with spontaneous aortic arch thrombosis mimicking aortic coarctation. Despite single case reports until now, no data exist for the underlying thrombophilic risk factors and prognosis of this rare event. Both patients were carriers of a heterozygous factor V Leiden mutation, which has been reported once before as a risk factor for aortic arch thrombosis. One of our patients was operated upon successfully and is alive. The second patient suffered a large infarction of the right medial cerebral artery and had a thrombotic occlusion of the inferior caval vein. The patient obtained palliative care and died at the age of 6 days. In the literature, we identified 19 patients with neonatal aortic arch thrombosis. Of the 19 patients, 11 (58%) died. Including the two reported patients, the mortality rate of patients with multiple thromboses was 80% (8/10) compared with 18% (2/11) for patients with isolated aortic arch thrombosis; this difference reached statistical significance (p = 0.009). The analysis of thrombophilic disorders revealed that factor V Leiden mutation and protein C deficiency seem to be the most common risk factors for aortic arch thrombosis. CONCLUSION: Neonatal aortic arch thrombosis is a very rare but life-threatening event, with a high rate of mortality, especially if additional thrombotic complications are present. Factor V Leiden mutation seems to be one important risk factor in the pathogenesis of this fatal disease.

Surgical management of large tracheoesophageal fistula in infants after button battery ingestion.

OBJECTIVES: In recent years, an increase in severe and even fatal outcomes related to oesophageal or airway button battery (BB) ingestion by infants and small children has been reported. Extensive tissue necrosis caused by lodged BB can lead to major complications, including tracheoesophageal fistula (TEF). In these instances, best treatment remains controversial. While small defects may warrant a conservative approach, surgery often remains inevitable in highly complex cases with large TEF. We present a series of small children that underwent successful surgical management by a multidisciplinary team in our institution. METHODS: This is a retrospective analysis of n = 4 patients <18 months undergoing TEF repair from 2018 to 2021. RESULTS: Surgical repair under extracorporeal membrane oxygenation (ECMO) support was feasible in n = 4 patients by reconstructing the trachea with decellularized aortic homografts that were buttressed with pedicled latissimus dorsi muscle flaps. While direct oesophageal repair was feasible in 1 patient, 3 required esophagogastrostomy and secondary repair. The procedure was completed successfully in all 4 children with no mortality and acceptable morbidity. CONCLUSIONS: Tracheo-oesophageal repair after BB ingestion remains challenging and is associated with major morbidity. Bioprosthetic materials in conjunction with the interposition of vascularized tissue flaps between trachea and oesophagus appear to be a valid approach to manage severe cases.

Indications and outcome after lung transplantation in children under 12 years of age: A 16-year single center experience.

OBJECTIVE: Paediatric lung transplantation poses unique management challenges. Experience regarding indications and outcome is scarce, especially in younger children. The primary aim of this study was to investigate outcome after first lung transplantation in children <12 years of age in comparison to adolescents (12-17 years old). METHODS: Records of patients <18 years who underwent first lung transplantation between 01/2005 and 01/2021 were retrospectively reviewed, and compared between children <12 years old and adolescents. Median (IQR) follow-up was 51 (23-91) months. RESULTS: Of the 117 patients underwent first lung transplantation at our institution, of whom 42 (35.8%) patients were <12 years and 75 (64.2%) ≥12 years old. Compared to adolescents, children were more often transplanted for interstitial lung disease (33.3% vs 12%, p = 0.005) and precapillary pulmonary hypertension (28.6% vs 12%, p = 0.025), and required more often intraoperative cardiopulmonary bypass (31% vs 14.7%, p = 0.036) and postoperative ECMO support (47.6% vs 13.3%, p < 0.001). Postoperatively, children required longer ventilation times (78 vs 18 hours, p = 0.009) and longer ICU stay (9.5 vs 3 days, p < 0.001) compared to their older counterparts. Primary graft dysfunction grade 3 at 72 hours (9.5% vs 9.3%, p = 0.999), in-hospital mortality (2.4% vs 6.7%, p = 0.418), graft survival (80% vs 62%, p = 0.479) and freedom from chronic lung allograft dysfunction (76% vs 59%, p = 0.41) at 8-year follow-up did not differ between groups. CONCLUSIONS: Lung transplantation in children under 12 years is challenging due to underlying medical conditions and operative complexity. Nevertheless, outcomes are comparable to those in older children.