From 1973 to 1986, 50 infants with sagittal synostosis have been operated by three different methods of craniectomy (linear craniectomy and extended craniectomies, as proposed by Schut and Epstein et al.). Preoperatively, the mean cephalic index was 67 +/- 4, 35.5% had clinical findings as cerebral palsy, psychomotor retardation and/or neurological signs, and intraoperatively the epidural pressure was more than 200 mm H2O in 60% (recorded in the last 20 patients). The mean follow-up time was 4.7 (1-10.6) years. Postoperatively, only 14.5% had minor clinical signs, which were mostly not in relation to the former scaphocephaly. Half of the patients with increased ICP had clinical signs preoperatively, and none of the 20 patients had distinct findings postoperatively. Out of the 20 children operated on by linear craniectomy or by Schut's method up to 1980, two-thirds had no school problems and one-third some school problems; one-third had occasionally headaches and one-quarter ametropia. Concerning the aesthetic results, Epstein's method and, somewhat less Schut's method, were superior to linear craniectomy, as verified by craniometry and by the tracings of the outlines of the neurocranium 0.4-0.7 and 1.6-2.0 years postoperatively: mean cephalic indices 73 +/- 5 (normal in one-fourth), 74 +/- 7 (normal in half) and 79 +/- 4 (normal in nearly all patients). Epstein's method is superior to the other two methods because it renders it possible to increase the breadth the greatest during the period of greatest postnatal brain growth. In addition to the effect on the neurocranium, the extended craniectomies add to normalization of the base of the skull (in contrast to the natural history of scaphocephaly). In the long run, the results obtained remain the same. The disadvantage of residual skull defects (approximately 11% of the patients with extended craniectomies) can be avoided by performing surgery prior to 4-6 months of age or by preserving the removed bone in a deep-freeze for a limited time.

Methods: In order to assess intermediate-term speech outcome after pharyngeal flap surgery for velopharyngeal dysfunction in children with cleft palate between 1980 and 1998, their pre- and postoperative speech performance was analyzed in a blinded fashion by speech pathologists and adult lay people. Speech was evaluated on the basis of tape recordings with regard to resonance, intelligibility, articulation, voice and secondary speech disorders. Results: Twenty-three patients could be evaluated. Both lay assessors and speech pathologists noted a significant improvement in speech performance after pharyngeal flap surgery. The percentage of children who improved was 83% (19/23, 95% confidence interval: 0.68–0.98, p = 0.002) when rated by lay people, and 87% (20/23, CI 0.73–1.01, p < 0.0001) when rated by professionals. Rated on a 5-point scale, the mean improvement per speech characteristic was 0.52 ± 0.32 scale points when judged by lay people, and 0.75 ± 0.8 points when judged by experts. Experts considered none of the children to have normal speech after surgery. Agreement with regard to outcome between lay people and speech pathologists occurred in 87% of the patients. Conclusion: The cranially based pharyngeal flap can improve speech performance in cleft palate children with chronic velopharyngeal insufficiency. However, it cannot be expected that this type of surgery will result in normal speech.

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Day-case surgery involves a considerable amount of stress not only for the children who undergo surgery but also for their parents. In a prospective survey analysis performed in 1995/1996, we identified the following main factors influencing the amount of stress experienced by parents: feelings of insufficient preparation and problems with postoperative pain at home. As a consequence, measures were taken regarding information and pain management, including the creation of an interactive CD-ROM. We then analysed the consequences of our interventions regarding parental stress. In this study we collected the data of all patients who underwent day-case surgery during two additional 13-month periods in 1997/1998 and 1999/2000. The methods of data collection remained unchanged for all three periods and included a questionnaire for the parents. In total, 1,490 questionnaires were analysed. Comparing the three time periods, parents' feelings of being well-informed improved significantly (91% vs. 98% vs. 97%, P < 0.0001). However, the percentage of those experiencing moderate to severe stress did not change substantially (16% vs. 9% vs. 19%, not significant). Analgaesics were given more frequently over the years in a general as well as a prophylactic manner (20% vs. 35% vs. 43%, P < 0.0001). Nevertheless, problems with pain control at home remained unchanged (33% vs. 23% vs. 29%, not significant). Despite considerable efforts to improve information, parental stress did not significantly decrease. It cannot be assessed yet whether wrong information was given or whether a certain degree of stress cannot be prevented. There is some evidence that improvements in coping with pain-related problems might be a promising next step.

The aim of the study was to analyse the outcomes of children born with oesophageal atresia over the last 3 decades. The records of 104 patients born between 1973 and 1999 were reviewed retrospectively. To evaluate changes over time, the analysis was done for three consecutive time periods: 1973-79, 1980-89, and 1990-99. Mean birth weight was 2553 g (SD 640), and mean gestational age was 39 weeks (SD 4). Forty-two newborns (40%) had one or more associated congenital malformations, and 30% had associated cardiac malformations. There was no change in incidence of associated anomalies over the three time periods studied. Mortality of patients decreased from 33% to 14% (p = 0.048). There was a significant association between the presence of a major cardiac malformation and survival (survival: 88% vs. 57%, p = 0.004). Analysing the three different time periods separately reveals that cardiac disease was not a significant risk factor in the first period but became significant in the period from 1980-99 (relative risk: 6.76, 95% CI 1.44-31.77). Birth weight was significantly higher in infants who survived (2626 g, SD 642) compared with those who died (2290, SD 570, p = 0.028). This effect, however, is mainly based on the difference during the first period and is lost later. Early and late postoperative complications occurred in 44/102 patients. Strictures developed in 33/91 patients who survived the first month of life (33%). The rate of symptomatic strictures decreased significantly over the three time periods, from 50% to 23% (p = 0.022). In summary, this study shows no significant change in patient characteristics over the last 3 decades, but mortality and postoperative complication rates decreased, and associated cardiac anomalies became the far most important risk factor for mortality.

10 children with Dandy-Walker syndrome are presented to discuss their clinical assessment and therapy. The presence on physical examination of a large posterior fossa was the most reliable clinical finding and was diagnostic in 9 out of the 10 patients. The different diagnostic confirmatory studies are discussed. The most satisfactory treatment in this series was the combination of a shunt from the lateral ventricle and the posterior fossa to the peritoneum.

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