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High survival in adult patients with acute respiratory distress syndrome treated by extracorporeal membrane oxygenation, minimal sedation, and pressure supported ventilation

AbstractObjectives: To evaluate the results of treatment of severe acute respiratory distress syndrome (ARDS) with extracorporeal membrane oxygenation (ECMO), minimal sedation, and pressure supported ventilation.n Designandsetting: Observational study in a tertiary referral center, Intensive Care Unit, Astrid Lindgren Childrens Hospital at Karolinska Hospital, Stockholm, Sweden.n Subjectsandmethods: Seventeen adult patients with ARDS were treated with venovenous or venoarterial ECMO after failure of conventional therapy. The Murray score of pulmonary injury averaged 3.5 (3.0–4.0) and the mean PaO2/FIO2 ratio was 46 (31–65). A standard ECMO circuit with nonheparinized surfaces was used. The patients were minimally sedated and received pressure-supported ventilation. High inspiratory pressures were avoided and arterial saturation as low as 70% was accepted on venovenous bypass.n Results: In one patient a stable bypass could not be established. Among the remaining 16 patients 13 survived (total survival rate 76%) after 3–52xa0days (mean 15) on bypass. Major surgical procedures were performed in several patients. The cause of death in the three nonsurvivors was intracranial complications leading to total cerebral infarction.n Conclusion: A high survival rate can be obtained in adult patients with severe ARDS using ECMO and pressure-supported ventilation with minimal sedation. Surgical complications are amenable to surgical treatment during ECMO. Bleeding problems can generally be controlled but require immediate and aggressive approach. It is difficult or impossible to decide when a lung disease is irreversible, and prolonged ECMO treatment may be successful even in the absence of any detectable lung function.

Inter-hospital transportation of patients with severe acute respiratory failure on extracorporeal membrane oxygenation – national and international experience

Abstract.Objective: To evaluate the experiences and results from inter-hospital transportation of patients with acute respiratory failure on extracorporeal membrane oxygenation (ECMO). Design: Observational, descriptive study. Setting: Tertiary referral center in a University Hospital. Subjects and methods: When standard ECMO criteria were fulfilled and the patient considered too unstable for a conventional transport, the mobile ECMO team cannulated the patient for ECMO at the referring hospital. The patients were then transported to our ECMO center by ground ambulance, helicopter or fixed-wing vehicle. Patients were also transported on ECMO from our ECMO center to other centers due to shortage of available ECMO beds. Results: 29 patients (15 neonates, seven pediatric, and seven adult patients) with acute respiratory failure were transported on ECMO on a total of 30 occasions. Median time from arrival of the ECMO team at the referring hospital until the patient was on ECMO (28 patients) was 2.2xa0h (range 1.25–4.25xa0h). The median time that the transport team was out was 10xa0h (range 5.5–36.5xa0h) and the median time with the patient was 6xa0h (range 3–30.5xa0h). The distance of transport ranged from 4–1,500xa0km. Six transports were international. No patient complications occurred during the transports. Two technical complications related to the transport vehicle were encountered. One ambulance compressor malfunctioned. During one helicopter transport, one out of two electric supply circuits malfunctioned. The patients were not affected. Twenty-one of the 29 patients survived to discharge (72%). None of the deaths was transport related. Conclusions: Tertiary intensive care units and ECMO centers require a dedicated transport team. ECMO transports can be performed safely for all age groups for long distances, probably throughout most of Europe.

Congenital diaphragmatic hernia: a survey of practice in Scandinavia

There is no consensus on the treatment of congenital diaphragmatic hernia (CDH), and practice seems to vary between centres. The main purpose of the present study was to survey current practice in Scandinavia. Thirteen paediatric surgical centres serving a population of about 22 million were invited, and all participated. One questionnaire was completed at each centre. The questionnaire evaluated management following prenatal diagnosis, intensive care strategies, operative treatment, and long-term follow-up. Survival data (1995–1998) were available from 12xa0ofxa013xa0centres. Following prenatal diagnosis of CDH, vaginal delivery and maternal steroids were used at eight and six centres, respectively. All centres used high-frequency oscillation ventilation (HFOV), nitric oxide (NO), and surfactant comparatively often. Five centres had extracorporeal membrane oxygenation (ECMO) facilities, and four centres transferred ECMO candidates. The majority of centres (7/9) always tried HFOV before ECMO was instituted. Surgery was performed when the neonate was clinically stable (11/13) and when no signs of pulmonary hypertension were detected by echo-Doppler (6/13). The repair was performed by laparotomy at all centres and most commonly with nonabsorbable sutures (8/13). Thoracic drain was used routinely at seven centres. Long-term follow-up at a paediatric surgical centre was uncommon (3/13). Only three centres treated more than five CDH patients per year. Comparing survival in centres treating more than five with those treating five or fewer CDH patients per year, there was a tendency towards better survival in the higher-volume centres (72.4%) than in the centres with lower volume (58.7%), p =0.065.

Platelet-derived growth factor inhibition—a new treatment of pulmonary hypertension in congenital diaphragmatic hernia?

Increased pulmonary vascular resistance causing pulmonary artery hypertension is a major problem in the treatment of congenital diaphragmatic hernia with a strong association to mortality. We here report a patient with intractable pulmonary hypertension at 4 weeks of age unresponsive to conventional treatment. After administration of the platelet-derived growth factor (PDGF) receptor antagonist imatinib, pulmonary artery pressure gradually decreased to acceptable levels and the patients clinical condition gradually improved.

Translation and cultural adaptation of the Hirschsprung’s Disease/Anorectal Malformation Quality of life Questionnaire (HAQL) into Swedish

AbstractPurposenChildren with anorectal malformation or Hirschsprung’s Disease (HD) often have functional problems with constipation or incontinence. The Hirschsprung’s Disease/Anorectal malformation Quality of life Questionnaire (HAQL) developed in the Netherlands is a disease-specific instrument measuring the quality of life (QoL) of children and adolescents with fecal incontinence. HAQL includes several domains with questions concerning diet, laxatives, constipation, diarrhea, urine and fecal incontinence, in addition to social and emotional functioning, body image, and physical symptoms. The purpose of the study was to translate and culturally adapt the HAQL questionnaire into Swedish.MethodThe translation was carried out according to accepted translation guidelines and a backward/forward translation method was used.ResultsThe translation correlated well with the original. All in all the Swedish and the Dutch versions agreed well. The Swedish translators chose to use a more simplified language in the questionnaires intended for the children, but used another choice of words in the proxy version and the adolescents’ version.ConclusionsThe translation of the HAQL instrument into Swedish gives us a disease-specific QoL instrument for children and adolescents born with HD and anorectal malformations (ARM). The translated and culturally adapted HAQL instrument is included in a survey regarding children and adolescents born with ARM.

Parental Experiences : Care of Children With High and Intermediate Imperforate Anus

In this study parental experiences of care of children with high and intermediate imperforate anus were evaluated. A group of 45 parents of children with high and intermediate imperforate anus and two control groups participated. Data collection with individual questionnaires concerning the childs hospital care, information to the parent and the child, and involvement in the care of the child were performed. Parents of children with imperforate anus reported being less satisfied with the care of their child, and they were less content with information about their childs treatment compared with the control groups. The parents had been extremely involved in the follow-up treatment. Constipation and fecal incontinence are common and involve suffering for the children and their parents. Parents have to be motivated and supportive and have a great deal of patience to be able to put up with caring for these children, and it seems as if health care professionals have underestimated their problems.

Results of Surgical Treatment of Lung Metastases in Children

Between 1970 and 1979, 14 patients were operated on for pulmonary metastases. In 9 of these the primary tumour was a Wilms nephroblastoma. Five out of the 14 patients are presently alive and free from disease (3 to 10 years after pulmonary resection). Primary tumours of the survivors were Wilms tumour in 3 cases, an ovarian teratoma in one patient and a Ewings sarcoma in one patient. Two of survivors had bilateral involvement and one had multiple metastases of one lung with pleural exudation. The disease-free interval was the same among the survivors and the non-survivors. It is apparent from the present material that some patients with a very poor prognosis as predicted from different prognostic factors can be cured after surgical excision of the metastases. The surgical procedure in children involves a very small risk and only moderate discomfort. An active surgical approach is therefore recommended.

Children with high and intermediate imperforate anus: their experiences of hospital care

PurposeThe purpose of this study was to examine the experiences of children with high and intermediate imperforate anus (IA), and specifically their experiences of hospital care.MethodsTwenty-five children born with high and intermediate IA participated; 9 boys and 16 girls. The mean age was 10.5xa0years (range 8.0–13.6). Two control groups were involved in the study. A self-report questionnaire was used to gather the data concerning children’s experiences of hospital care. Items were scored on a 5-point Likert scale.ResultsThe children’s responses on hospital care items scored high. The children with IA reported being less satisfied with the information given, and understood less why they needed to visit the hospital than did the children in the two control groups.ConclusionThe children’s experiences of care seemed to be positive even though the children born with IA are subjected to invasive treatment. More research is called for in the unexplored area of information to the children, and particularly to the children born with a defect. Children’s views are important and should always be considered, as their answers most certainly reflect a genuine experience, contributing to the further development of their specific care.