Oesophageal atresia and tracheo-oesophageal fistula.

Oesophageal atresia-tracheo-oesophageal fistula has featured in paediatric surgery since its beginnings. The first successful primary repair was in 1941. With overall survival now exceeding 90% in dedicated centres, the emphasis has changed to reducing morbidity and achieving improvements in the quality of life.

Non-epileptic clinical diagnoses in children referred for an outpatient EEG using video monitoring.

Simultaneous video (closed circuit television [CCTV]) and EEG recordings are important in the differentiation of epileptic and non-epileptic paroxysmal episodes and in the classification of epilepsy syndromes. An additional benefit from the observation of the child on CCTV is the possible identification of specific clinical, including genetic, conditions. This three-year prospective study of 2780 consecutive children undergoing routine EEG investigations identified 17 conditions that had not previously been diagnosed by the clinicians who had requested the EEG.

Perinatal management of congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) retains high mortality due to lung hypoplasia and pulmonary hypertension. Efforts to improve survival and outcome have included fetal intervention, delivery at specialist centres, elective operation after stabilisation of labile physiology and minimising barotrauma. Permissive hypercapnea ('gentle ventilation') represents a significant advance in therapy gaining wider acceptance in centres worldwide.

Secondary pulmonary hypertension.

Recent research has greatly improved our understanding of the pathophysiology of pulmonary hypertension. There is increasing recognition that pulmonary hypertension is an important complication of many childhood respiratory diseases including cystic fibrosis, interstitial lung diseases, upper airways obstruction and disorders of the respiratory muscles and chest wall. Chronic hypoxaemia and, in some cases, destruction of the vascular bed are the key factors.

The influence of hyperchloraemia on acid base interpretation in diabetic ketoacidosis.

Objectives: During the acute treatment of diabetic ketoacidosis we (a) determined the temporal incidence of hyperchloraemia, and (b) quantified the influence of hyperchloraemia on interpretation of common blood gas derived acid base parameters, namely base deficit and bicarbonate.

Design And Setting: Retrospective chart review in two regional paediatric intensive care units.

Measurements And Results: Stewart's physicochemical theory was used to develop regression equations quantifying the acidifying effect of hyperchloraemia on both base deficit and bicarbonate.

British Association of Paediatric Otorhinolaryngology members experience with recurrent respiratory papillomatosis.

Objectives: To establish current practice in the treatment of recurrent respiratory papillomatosis (RRP) in the UK.

Methods: Consultant members of the British Association of Paediatric Otorhinolaryngology (BAPO) were sent a questionnaire on current practice concerning the management of recurrent respiratory papillomatosis in the paediatric population.

Results: The response rate was 39.

Iatrogenic iodine as a cause of hypothyroidism in infants with end-stage renal failure.

Between 1996 and 2005, two of seven infants in our unit on overnight continuous-cycle peritoneal dialysis (CCPD) acquired hypothyroidism following normal thyroid function on neonatal screening. Case 1 had posterior urethral valves, commenced CCPD at day 29, and developed hypothyroidism requiring treatment at 3 months: TSH 258 micro/l (ref.: 0.

Sex and congenital diaphragmatic hernia.

Background And Purpose: Human studies note sex reversal syndromes and sex difference(s) in the incidence of congenital diaphragmatic hernia (CDH). Epidemiology surveys record a higher incidence of CDH in females, whilst other reports cite a higher frequency in males. Nitrofen, a teratogen, produces experimental CDH.

Management of craniofacial abnormalities.

A number of congenital and acquired conditions can affect the skull, face and jaws resulting in a wide range of craniofacial abnormalites that commonly present at birth or in early infancy. This article aims to outline the aetiology, pathogenesis, diagnosis and principles of management of those conditions that commonly present to a craniofacial unit namely the craniosynostoses.

Transillumination of H-type tracheoesophageal fistula using flexible miniature bronchoscopy: an innovative technique for operative localization.

Precise localization of the fistula is the most important step in the operative strategy for dealing with H-type tracheoesophageal fistula. Bronchoscopic cannulation of the fistula with a Fogarty or ureteric catheter has been recommended to aid ready identification, but it is not always successful. We report an innovative technique that permitted localization of H-type fistula intraoperatively.

An unusual case of Turcot's syndrome associated with ileal adenocarcinoma, intestinal non-Hodgkin's lymphoma, and duodenal adenocarcinoma. Review of the classification and genetic basis of Turcot's syndrome.

A 38-year-old man with a history of colonic and small bowel polyposis and glioblastoma was investigated for dyspepsia. Upper GI endoscopy identified an abnormal area in the duodenum, confirmed by histology as high grade non-Hodgkin's B cell MALT lymphoma. Although cases of Turcot's syndrome (TS) (colonic polyposis and primary brain tumour occurring in the same patient) have been previously described, association with haematological malignancy is rare.

Paediatric otolaryngology services in the UK: a postal questionnaire survey of ENT consultants.

Approximately half a million children in England and Wales receive in-patient or day-case surgical treatment annually. Otolaryngology is the surgical specialty that provides the greatest number of episodes of such care. As 30-50 per cent of our total volume of work is paediatric, we feel it is important to assess current attitudes to paediatric otolaryngological practice.

Spontaneous propagating calcium waves underpin airway peristalsis in embryonic rat lung.

Prenatal airways from diverse species exhibit spontaneous peristaltic contractions (airway peristalsis). These contractile waves appear coupled to and may function to regulate prenatal lung growth. They are unaffected by atropine or tetrodotoxin but abolished by nifedipine.

Recent advances in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a common birth defect which continues to challenge paediatric surgeons and intensivists. Affecting approximately 1:2500 births, a baby with CDH is born every 24-36 hours in the UK.

Emergent embolisation to control severe haematuria in Wilms' tumour.

Wilms' tumour commonly presents with an abdominal mass and gross haematuria. Here, we present the novel application of paediatric renal arterial embolisation to control life-threatening haematuria in Wilms' tumour.

Index finger abnormalities in Simpson-Golabi-Behmel syndrome.

Simpson-Golabi-Behmel syndrome (SGBS) is an X linked recessive overgrowth disorder in which digital abnormalities are a well-described aspect of the phenotype. We report a case with marked index finger hypoplasia and a congenital abnormality of the proximal phalanx and review the literature detailing index finger abnormalities in this condition.

Multivariate analysis on factors affecting suppression of thyroid-stimulating hormone in treated congenital hypothyroidism.

Aims: To determine the factors which influence the suppression of thyroid-stimulating hormone (TSH) in infants with congenital hypothyroidism (CH) following treatment.

Methods: We examined retrospectively the patterns of thyroid function tests from diagnosis to 3 years of age in 140 infants diagnosed with CH from screening. Patients were classified into 3 groups: athyreosis, ectopia and presumed dyshormonogenesis on the basis of thyroid scans.

Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases?

Background: The majority of congenital cystic adenomatoid malformation (CCAM) lesions are diagnosed antenatally. A few cases however may not be recognised antenatally and present in infancy or later childhood with chest symptoms, including chest infection.

Objective: To review the clinical and radiological spectrum of CCAM, comparing the antenatally with the postnatally diagnosed cases.

Febrile seizures: an update.

This review focuses on the latest knowledge and understanding of febrile seizures and outlines the more important issues in the management of children who present with an apparent "febrile seizure". It is not the remit of this paper to discuss the detailed management of febrile seizures. Throughout this review, the words "partial" and "focal" will be used interchangeably and the term "febrile seizure" (FS) will be used, reflecting the proposed changes in the terminology of seizures and epilepsies.

RSV outbreak in a paediatric intensive care unit.

The Royal Liverpool Children's Hospital-Alder Hey paediatric intensive care unit (PICU) usually has a low rate of nosocomial respiratory syncytial virus (RSV) infection. We report and analyse a major outbreak of nosocomial (acquired) RSV infection on the PICU during a RSV season. All children admitted to the PICU were studied during the six-month winter period 1 October 2002 to 31 March 2002.

Head circumference and linear growth during the first 3 years in treated congenital hypothyroidism in relation to aetiology and initial biochemical severity.

Aims: To determine the head circumference and linear growth in children with congenital hypothyroidism (CH) during the first 3 years of life in relation to the aetiology of CH and initial biochemical severity of thyroid function.

Methods: We examined the head circumference and linear growth of 125 patients with CH from diagnosis up to 3 years of age. All infants had radionuclide scans prior to treatment.

Distal arthrogryposis type IIB: unreported ophthalmic findings.

We describe four members spanning three generations of a Caucasian family affected with distal arthrogryposis (DA). Based on Hall's original classification, we have placed our family under type IIB and present previously unreported ophthalmic features. All the members had different degrees of ophthalmoplegia, ptosis, astigmatism, and strabismus.

Managing sedation withdrawal in children who undergo prolonged PICU admission after discharge to the ward.

Children who undergo a prolonged stay within the intensive care unit require adequate sedation and analgesia. During the recovery phase there will need to be a period of sedation withdrawal to prevent occurrence of an abstinence syndrome. We present a strategy developed within our hospital for managing this process which uses the resource of the Pain Service, along with guidelines to help prevent the development of withdrawal, and a plan for managing any signs of abstinence which occur.