Long-Term Outcomes and Health-Related Quality of Life in a Swiss Patient Group with Esophageal Atresia.

INTRODUCTION: Surgical treatment of esophageal atresia (EA) has markedly improved, allowing the focus to shift from short-term complications and mortality to long-term complications and quality of life. Health-related quality of life (HRQoL) is variable and reported to range from reduced to unimpaired in patients with repaired EA. We assessed the HRQoL, determined the prevalence of long-term complications and their possible impact on the HRQoL in patients who had correction of EA in Switzerland. Further, we also investigated in the general well-being of their parents. MATERIALS AND METHODS: Patients with EA repair in Switzerland between 1985 and 2011 were enrolled. Long-term complications were assessed by enquiring disease-related symptoms, standardized clinical examinations, and analysis of radiographs. HRQoL was inquired using different validated questionnaires (KIDSCREEN-27, World Health Organization [WHO]-5, and Gastrointestinal Quality of Life Index [GIQLI]). Patients were grouped according to their age. In underage patients, general well-being of the parents was assessed using the WHO-5 questionnaire. RESULTS: Thirty patients were included with a mean age of 11.3 ± 5.7 years. Long-term complications were present in 63% of all patients. HRQoL in underage patients was comparable to the provided reference values and rated as good, while adult patients reported a reduced HRQoL. The presence of gastroesophageal reflux disease symptoms was associated with reduced HRQoL in underage patients. Parents of underage patients stated a good general well-being. CONCLUSION: Long-term complications among patients with repair of EA in Switzerland are common. HRQoL in underage patients is good and general well-being of their parents is unimpaired. Adult patients reported a reduced HRQoL, consistent with other reports. As long-term complications may manifest only later in life, a structured follow-up of patients with an EA repair during childhood and adolescence is needed.

Abdominal compartment syndrome and decompressive laparotomy in children: a 9-year single-center experience.

PURPOSE: Abdominal compartment syndrome (ACS) in children results in 100% mortality if left untreated. Decompressive laparotomy (DL) is the only effective treatment if conservative medical therapies have failed. This study aims to determine the incidence of ACS among pediatric patients who underwent an emergency laparotomy (EL), to describe the effect of DL on clinical and laboratory parameters and, to make a better prediction on fatal outcome, to analyze variables and their association with mortality. METHODS: This retrospective study includes 418 children up to the age of 16 years who underwent EL between January 2010 and December 2018 at our tertiary pediatric referral center. ACS was defined according to the latest guidelines of the World Society of the Abdominal Compartment Syndrome. RESULTS: Fourteen patients had emergency DL for ACS. 6 h preoperatively; median intra-abdominal pressure (IAP) and abdominal perfusion pressure (APP) were 22.5 mmHg and 29 mmHg, respectively. After DL, IAP decreased and APP increased, both by an average of 60%. Six patients survived, eight patients had a fatal outcome, resulting in a mortality of 57%. An age under 1 year, weight under the 3rd percentile, an open abdomen treatment, an intestinal resection and an elevated serum lactate > 1.8 mmol/L were associated with an increased relative risk of death. CONCLUSIONS: Improving the outcome in pediatric patients with ACS by removing or attenuating risk factors is difficult. This emphasizes the need for early diagnosis and prompt DL once the diagnosis of ACS is made.

Correction to: Compartment pressures in children with normal and fractured lower extremities.

The original version of this article unfortunately contained a mistake in the author name Sasha Job Tharakan. The corrected name is given above.

Compartment pressures in children with normal and fractured lower extremities.

PURPOSE: Needle manometry is a tool to confirm suspected acute compartment syndrome (ACS). There is scarce evidence of normal pressure values of the lower extremities in children. The aim of this study is to assess the normal compartment pressures in non-injured lower extremities of children. METHODS: This prospective study included children up to the age of 16 years with lower extremity fractures that needed reduction. Between June 2009 and August 2015, 20 children were included. We used needle manometry to measure the pressures in the superficial (SPC), deep posterior (DPC) and in the anterior compartments (AC) on both the lower legs. RESULTS: On the healthy leg, the mean compartment pressure was 15.15 mmHg in the AC (range 7-30 mmHg), 14.32 mmHg in the SPC (range 8-24 mmHg) and 13.00 mmHg in the DPC (range 4-21 mmHg). On the injured leg, the mean compartment pressure was 24.07 mmHg in the AC (range 5-40 mmHg), 17.21 mmHg in the SPC (range 7-29 mmHg) and 17.13 mmHg in the DPC (range 6-37 mmHg). We found a perfusion gradient (diastolic blood pressure-compartment pressure) < 30 mmHg in at least one compartment of the fractured and healthy leg in 13 patients. Five patients underwent fasciotomy for suspected ACS and their data was excluded for the injured leg. CONCLUSION: We could show that children have higher normal compartment pressures than adults in the lower leg. They seem to be able to tolerate higher absolute compartment pressures and lower pressure gradients before ACS occurs. More studies are needed to make a final statement on tolerable compartment pressures in children.

Comparison of lower extremity fasciotomy wound closure techniques in children: vacuum-assisted closure device versus temporary synthetic skin replacement.

PURPOSE: No clear consensus on the optimal treatment of fasciotomy wounds due to acute compartment syndrome of the lower leg in children exists. We therefore compared two commonly used methods to close fasciotomy wounds, Epigard, a temporary synthetic skin replacement (SSR) and the vacuum-assisted closure (V.A.C.) device, in respect of treatment duration and complication rates. METHODS: We studied the cases of 27 patients who were treated at our institution for acute compartment syndrome of the lower leg with a fasciotomy over a 10-year period. The fasciotomy wound was either treated with SSR or V.A.C. device. We recorded the number of procedures to definitive wound closure, days to wound closure, hospitalization days and sequelae rate. RESULTS: In the V.A.C. device group (18 patients) the mean number of procedures until definitive wound closure was 3.1, mean days until wound closure was 9.4 and mean days of hospitalization was 16.2. One patient suffered from a wound infection and one patient required a full thickness skin graft. In the SSR group (9 patients), the mean number of procedures was 1.8, mean days until definitive wound closure was 4.9 and mean days of hospitalization was 9.9. No sequelae were recorded. There was a statistically significant smaller number of procedures (p value 0.018), fewer days to definitive wound closure (p value 0.002) and fewer hospitalization days (p value 0.005) in the SSR group. CONCLUSIONS: Both SSR and V.A.C. device are safe and reliable for closure of fasciotomy wounds in children, whereas SSR seems to lead to shorter time until definitive wound closure.

Hydroxyapatite ceramic implants for cranioplasty in children: a single-center experience.

PURPOSE: The use of hydroxyapatite ceramic (HAC) implants for the treatment of skull defects in pediatric patients started 2010 at our institution. Ceramic implants facilitate osteoblast migration and therefore optimize osteointegration with the host bone. The purpose of this study is to report a single-center experience with this treatment modality. METHODS: A retrospective review of all patients from July 2010 through June 2014 undergoing a cranioplasty using hydroxyapatite ceramic implant and managed at a single institution was performed. Indication for cranioplasty, the hospital course, and follow-up were reviewed. Bone density was measured in Hounsfield Units (HU) and osteointegration was calculated using Mimics Software® (Mimics Innovation Suite v17.0 Medical, Materialize, Leuven, Belgium). RESULTS: Over the 4-year period, six patients met criteria for the study. Five patients had an osteointegration of nearly 100%. One patient had an incomplete osteointegration with a total bone-implant contact area of 69%. The mean bone density was 2800 HU (2300-3000 HU). Bone density alone is estimated to have a Hounsfield value between 400 and 2000 HU depending on the body region and bone quality. There were no major complications, and the patients were highly satisfied with the esthetical result. CONCLUSION: Hydroxyapatite ceramic implants for cranioplasty in pediatric patients are a good choice for different indications. The implants show excellent osteointegration and esthetical results.