Prospective surveillance study of the management of intussusception in UK and Irish infants.

BACKGROUND: Intussusception is the most common cause of acute intestinal obstruction in infants. This study examined the clinical presentation, management and outcomes of intussusception in this age group. METHODS: Prospective surveillance of intussusception in infants was carried out between March 2008 and March 2009 in the UK and Ireland. Monthly cards were sent to paediatric clinicians who were requested to notify cases of intussusception. RESULTS: The study identified 261 confirmed cases. The commonest presenting symptom/sign was non-bilious vomiting, in 210 (80·5 per cent) of the infants. Abdominal ultrasonography was done in 247 infants (94·6 per cent) and was diagnostic in 242 (98·0 per cent), compared with plain abdominal X-ray, which was diagnostic in 33 (23·6 per cent) of 140 infants. Enema reduction was carried out in 240 (92·0 per cent) of the 261 infants; the majority (237, 98·8 per cent) had pneumatic reduction with a success rate of 61·2 per cent (145 of 237). Surgery was required in 111 infants (42·5 per cent); 92 operations were as a result of unsuccessful enema reduction, and the remaining 19 infants (17·1 per cent) had primary surgery. Forty-four infants (39·6 per cent of operations) needed a bowel resection. The majority of children (238, 91·2 per cent) recovered uneventfully; 21 (8·0 per cent) had sequelae, one child died (0·4 per cent), and the outcome was unknown for one infant. CONCLUSION: This study described current treatment patterns for intussusception in infancy; these represent a benchmark for improved standards of care for this condition.

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.

Autologous blood pleurodesis for pneumothorax complicating graft-versus-host disease-related bronchiolitis obliterans.

Bronchiolitis obliterans (BO) is a manifestation of chronic graft-versus-host disease (GVHD) after allogeneic haemopoietic stem cell transplantation. Complications associated with this include persistent air-leak syndromes such as pneumothorax. Many methods have been described for treating this condition, both surgical and nonsurgical. We describe an 8-year-old boy with acute lymphoblastic leukaemia complicated by chronic GVHD-related BO, and subsequent pneumothorax with persistent air leak, who was treated successfully with autologous blood pleurodesis.

Obliterative enteritis complicating graft versus host disease.

We report a 10-year-old male with Down's syndrome, who received a bone marrow transplant for acute lymphoblastic leukaemia. Subsequent acute graft-versus-host disease (GvHD) of the gut progressed to small bowel obstruction. At laparotomy, the small bowel appeared solid and contracted with no or minimal luminal patency. Although the caecum had a lumen, it was indistensible, and it was not possible to enter the terminal ileum. Histology of the obstructed bowel showed extensive necrosis of the mucosa, muscularis mucosa and submucosa of most of the small bowel wall, causing obliteration of the lumen. The changes were presumed to be related to post inflammatory atrophy. This extreme manifestation of GvHD could thus be called obliterative enteritis. Both cytomegalovirus and adenovirus were isolated from the patient. These viruses may have contributed to the severity of the intestinal GvHD.

Detection of caustic oesophageal injury with technetium 99m-labelled sucralfate.

BACKGROUND/PURPOSE: Radiolabelled sucralfate has been used to show the extent and severity of reflux oesophagitis, peptic ulceration, and inflammatory bowel disease. Endoscopy under general anaesthetic has been the preferred method to assess the injury after caustic ingestion. The aim of this study was to assess whether sucralfate has an affinity for the chemically injured oesophageal mucosa and, if so, to assess the accuracy of radiolabeled sucralfate as an indicator of presence and extent of oesophageal injury. METHODS: A prospective study was conducted of 22 patients with mean age of 30 months (range, 13 to 90) admitted consecutively with a history of caustic ingestion between January 1998 and January 2000. A sucralfate-labelled scan followed by endoscopic assessment of upper gastrointestinal tract with documentation of extent and grade of injury was performed in all patients within 24 hours of admission except the first 6 who underwent scan after the endoscopy. The sucralfate was labelled by the direct stannous reduction method. Oesophageal transit was studied by recording 120 images (64 x 64 matrix size) at 1 image per second while the child swallowed 5 mL of labelled sucralfate containing 2 to 3 MBq Technetium 99m. Retention of radiolabelled oesophageal activity was considered abnormal. RESULTS: The caustic substances ingested were household cleaners in 18, potassium permanganate in 3, and pool chlorine in 1. There were 11 scans that showed residual activity in the oesophagus, which correlated exactly with endoscopic findings. The other 11 patients had normal oesophageal mucosa, but 2 were found to be falsely positive on scanning. In 2 cases repeat sucralfate scan results correlated well with the healing process assessed endoscopically. CONCLUSIONS: The results indicate that technetium 99m sucralfate swallow is an accurate technique for assessing oesophageal injury after ingestion of caustic substances. In addition, it may be used to document healing.

Small bowel intussusception in celiac disease.

Intussusception is not a widely recognized complication of celiac disease and yet it is not rare. The authors report on 3 children with spontaneously resolving small bowel intussusception in association with celiac disease. Small bowel intussusception in a child with suspected celiac disease initially should be managed expectantly rather than by early surgical reduction. The finding of transient small bowel intussusception, either by contrast radiology or sonography, should prompt investigation for celiac disease.

A comparison of neonatal and adult lung impedances derived from EIT images.

An objective method of extracting respiratory data from lung images is presented, together with a technique for automatically generating regions of interest delineating the anterior and posterior regions of the lungs. The method is used to extract data on the change in lung impedance with frequency, and on calculated Cole parameters, from 19 normal neonates (gestational age 32 to 42 weeks) and 8 normal adults (age 21 to 82 years). A comparison of the impedance properties of neonatal and adult lungs was made. The variation of lung impedance with frequency in neonates, as derived from EIT images, is significantly different from that found for adults. The implications for a model of the electrical impedance of lung tissue are discussed.

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.

Ultrasonography aids decision-making in children with abdominal pain.

INTRODUCTION: Although regular clinical assessment of the acute abdomen is considered best practice, ultrasonography confirming the presence of appendicitis will add to the decision-making process. The aim of this study was to assess the accuracy of ultrasonography and its usefulness in diagnosing acute appendicitis in a regional paediatric surgical institution. METHODS: Retrospectively and in this order, radiology, theatre and histopathology databases were searched for patients who had presented with acute abdominal pain, patients who had undergone an appendicectomy and all appendix specimens over a two-year period. The databases were cross-referenced against each other. RESULTS: A total of 273 non-incidental appendicectomies were performed over the study period. The negative appendicectomy rate was 16.5% and the perforation rate 23.7%. Thirty-nine per cent of children undergoing an appendicectomy had at least one pre-operative ultrasound scan. Ultrasonography as a diagnostic tool for acute appendicitis in children had a sensitivity of 83.3%, a specificity of 97.4%, a positive predictive value of 92.1% and a negative predictive value of 94.0%. CONCLUSIONS: Ultrasonography is used liberally to aid in the decision making process of equivocal and complicated cases of acute appendicitis and it achieves good measures of accuracy. As a diagnostic tool it is unique in its ability to positively predict as well as exclude. A high negative predictive value suggests that more patients could be managed on an outpatient basis following a negative scan.

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.

Intestinal ulceration, obstruction, and haemorrhage in congenital syphilis.

Intestinal obstruction and bleeding are uncommon complications of congenital syphilis (CS). A VDRL-positive infant developed incomplete intestinal obstruction and rectal bleeding. Despite conservative management, his symptoms continued. At laparotomy, terminal ileal inflammation and stenosis were demonstrated. He underwent ileal resection and primary end-to-end anastomosis with resolution of his symptoms. Histopathological examination demonstrated heavy plasmacytic infiltration of the lamina propria and submucosa with ulceration of the mucosa, consistent with syphilitic ileitis. This report documents for the first time bleeding from ileal ulcers associated with intestinal obstruction in CS and highlights an unusual presentation of the disease.

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.