Congenital apocrine tumour: a rare scalp tumour.

We report a rare congenital scalp tumour with histological diagnosis of a congenital apocrine adenoma with features of a tubular adenoma. Following cranial CT and MRI, the tumour was serially excised. The macroscopic and microscopic appearances and management are discussed. To our knowledge, such a case has not been previously reported.

Reproducibility of electrical impedance tomographic spectroscopy (EITS) parametric images of neonatal lungs.

The reproducibility of electrical impedance tomographic spectroscopy (EITS) images of neonatal lungs have been investigated in 11 clinically stable babies. We have used the Sheffield Mark IIIa EITS system. An average inspiration frame was generated from the data frames associated with maximum inspiration. Frequency images were reconstructed from these frames. The frequency images were analysed to locate the pixel with the maximum change in the right lung field. The change was defined as the 614 kHz measurement relative to 9.6 kHz. A 3 x 3 pixel region of interest was centred at this point. The changes in impedance with frequency for this region of interest show good overall reproducibility between electrode applications for eight frequencies (95% limits of agreement +/- 28%). This reproducibility is improved (95% limits of agreement +/- 13%) by omitting the highest frequency (1.2 MHz) which is most subject to system noise. The parameters for the Cole model derived from data with the highest frequency omitted are less reproducible between electrode applications (95% limits of agreement, R/S +/- 0.83, fc +/- 81.6, RC +/- 0.52, SC +/- 0.39). We suspect that the parametric model used may have an effect on this. The signals recorded at the highest frequency (1.2 MHz) are a major source of variability. The reproducibility results are improved by omitting this frequency from the analysis.

Congenital lung malformations: an ongoing controversy.

INTRODUCTION: Congenital lung malformations are rare lesions that are most commonly diagnosed antenatally. Management of such lesions, particularly those that are asymptomatic, remains controversial. We undertook a survey to ascertain current practice of surgeons in the UK and Ireland. METHODS: All consultant members of the British Association of Paediatric Surgeons were asked to complete a survey on congenital lung malformations with respect to antenatal management, symptomatic and asymptomatic lesions, and operative techniques. RESULTS: Responses were received from 20 paediatric surgical centres and highlighted the ongoing variability in management of such lesions, particularly those that are asymptomatic. Twenty per cent of surgeons never resect an asymptomatic lesion and twenty-four per cent always do. The remainder intervene selectively, with size being the most commonly stated indication. Most resections are undertaken via thoracotomy although 35% of surgeons use thoracoscopy for some procedures. CONCLUSIONS: National data based on congenital anomaly registers are needed to determine the natural history of these malformations and to guide future management.

Neonatal haemochromatosis associated with gastroschisis.

We describe, to our knowledge, the first case of progressive neonatal liver failure due to neonatal haemochromatosis (NH) occurring in an infant with a gastroschisis and review the literature regarding these two conditions. A 1,665 g male infant with antenatally diagnosed gastroschisis was born with a severe coagulopathy, anaemia, thrombocytopenia, hypoglycaemia and jaundice. He developed progressive liver failure, complicated by necrotising enterocolitis. Serum ferritin was elevated at 1,459 microg/L. He died on day 40 and a limited post-mortem examination confirmed significant hepatic siderosis with fibrosis and cholestasis, and siderosis of the pancreas. Although no genetic aetiology for gastroschisis has been identified, an occasional inherited tendency has been observed. There is also evidence to support an autosomal recessive inheritance in NH.

Three 'silent' mastoid abscesses.

Acute mastoiditis with abscess formation is uncommon, its incidence being modified by modern antibiotic usage for acute suppurative otitis media (ASOM). It tends to occur in young children who have no previous history of ear disease. The diagnosis is a clinical one, and treatment requires hospital admission and some form of surgical drainage procedure, in order to prevent intracranial complications, eg, meningitis and intracerebral abscess.

Pulmonary function, exercise performance, and growth in survivors of congenital diaphragmatic hernia.

A cohort of survivors of congenital diaphragmatic hernia (CDH), with matched controls, was studied to assess growth, respiratory function, and exercise performance. Nineteen of 24 survivors from an 11 year period (79%) were compared with 19 matched controls. Subjects had detailed auxology, performed spirometry and cycle ergometry, and completed questionnaires about respiratory symptoms and exercise. There were no significant differences between the groups for height, weight, sitting height, head circumference, or body mass index expressed as SD scores. The mean (95% confidence interval) percentage predicted forced vital capacity (FVC) was 84.7% (79.1 to 90.3) in index cases and 96.5% (91.4 to 101.6) in controls (p < 0.01). There was no significant difference in total lung capacity. Expiratory flow rates corrected for FVC were also similar between groups, suggesting normal airway function relative to lung size. Mean maximum oxygen consumption in ml/kg/min was 40.1 (36.8 to 43.4) and 42.2 (38.5 to 45.8) in index and control cases. These differences were not significant. Index cases achieved a similar minute ventilation to controls by more rapid and shallower breathing. Index cases had lower perception of their own fitness and lower enjoyment of exercise, although habitual activity levels were similar. Survivors of CDH repair have reduced functional lung volumes, but normal airway function compared with matched controls. They have no growth impairment nor significant impairment of exercise performance, although they have more negative perceptions of their own fitness. They should be encouraged and expected to participate fully in sport and exercise.