Extracardiac atrial switch for anatomical repair in variants of ccTGA.

Successful anatomical repair in congenitally corrected transposition of the great arteries depends on adequate re-routing of the atrial venous pathways. Abnormal positioning of the heart or abnormal venous drainage, or both, can impair access to the atrial pathways and previous palliative procedures can further complicate the reconstruction required to achieve anatomical repair. We describe 2 patients in whom the systemic venous anatomy was such that atrial switch was more easily accomplished by extracardiac re-routing than with the conventional Senning procedure.

Improving adherence to GH therapy with an electronic device: first experience with easypod.

Endocrinology units in the UK and Eire have, in recent years, moved away from offering a narrow range of injection devices and products to patients starting growth hormone (GH) treatment, and now most (approximately 90%) offer some form of patient choice. Survey results from a unit in Birmingham, UK, have shown that it is not possible to predict which device will be selected by a given patient based on their sex, age or diagnostic category. Across the UK, there is, however, wide variability in the methods used to present the devices to patients, the time invested in reaching an informed decision and the range of devices and manufacturers offered.

Case report: early bilateral simultaneous cochlear implantation in a five-year-old following head injury complicated by acute bacterial meningitis.

Meningitis is an important cause of profound sensorineural hearing loss, especially in children. In this case, a five-year-old suffered a head injury complicated by bacterial meningitis and developed a profound hearing loss. Magnetic resonance imaging at four weeks following injury showed evidence of developing labyrinthitis ossificans and a decision was made to perform bilateral cochlear implantation at an early stage.

Unusual complication following trauma to a bone-anchored hearing aid: case report and literature review.

Objective: We report the second published case of a child with a serious traumatic injury involving the fixture and abutment of their bone-anchored hearing aid.

Method: Case reports and review of the world literature concerning unusual complications following trauma to bone-anchored hearing aids.

Results: A nine-year-old girl with Dubowitz syndrome sustained an intrusion injury of her bone-anchored hearing aid fixture and abutment following a fall.

A four year retrospective study of 1,062 patients presenting with maxillofacial emergencies at a specialist paediatric hospital.

Aims: To examine the nature of maxillofacial injuries that presented to the Birmingham Children's Hospital according to aetiology, incidence and characteristics of patients.

Methods: The maxillofacial unit at Diana Princess of Wales Birmingham Children's Hospital serves a catchment area of 5.2 million.

Changes in potassium concentration and haematocrit associated with cardiopulmonary bypass in paediatric cardiac surgery.

Introduction: A blood prime is frequently required for paediatric bypass surgery to maintain adequate haematocrit (Hct). However, stored blood can have high extracellular potassium levels and this study aims to investigate the effect of stored blood on the potassium concentration, both in the prime and subsequently in the patient after cardiopulmonary bypass (CPB) has been established. In neonatal surgery, the stored blood may be irradiated if there is a question of impaired immunity.

Innominate venous aneurysm presenting as a rapidly expanding mediastinal mass.

Thoracic venous aneurysms are extremely rare conditions. Only 14 innominate venous aneurysms have been reported in the literature. We report a case of a 13-year-old girl who presented with a mediastinal mass on chest roentgenogram, which was performed because the patient was postoperatively tachypneic after a routine appendectomy.

Hypoplastic thumb in Gorlin's syndrome.

Gorlin's syndrome or naevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder that predisposes to basal cell carcinomas of the skin, ovarian fibromas, and medulloblastomas. This condition is due to mutations in the Patched (PTCH) gene which maps to chromosome 9q22 and acts as a tumour suppressor gene. Gorlin's syndrome is characterized by the development of multiple jaw keratocysts and/or basal carcinomas.

Transcatheter recanalization of ligated main pulmonary artery.

A 12.5-year-old boy with tricuspid atresia and quadriplegic cerebral palsy presented with increasing cyanosis after previous palliation with a cavopulmonary shunt and ligation of the main pulmonary artery (MPA). Because of severe physical disabilities he was not considered suitable for Fontan completion.

Arterial switch operation in patients with Taussig-Bing anomaly--influence of staged repair and coronary anatomy on outcome.

Objective: This study evaluated the results of arterial switch operation and closure of ventricular defects (ASO+VSDc) for double outlet right ventricle with sub-pulmonary ventricular septal defect (Taussig-Bing anomaly).

Methods: Between 1988 and 2003, 33 patients (25 male, 76%) with Taussig-Bing anomaly underwent ASO+VSDc (median age 39 days, 1 day-2.1 years).

Fate of pulmonary arteries following Norwood Procedure.

Objective: This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions.

Methods: Between 1993 and 2004, 226 patients underwent Stage II following NP.

The Pediatric Early Warning System score: a severity of illness score to predict urgent medical need in hospitalized children.

Purpose: We developed and performed the initial retrospective validation of a pediatric severity of illness score. The score is to preemptively identify hospitalized children who are likely to require resuscitation to treat cardiopulmonary arrest.

Materials And Methods: The Pediatric Early Warning System (PEWS) score was developed using expert opinion.

Intracranial pressure complicating severe traumatic brain injury in children: monitoring and management.

Objective: To identify factors associated with the use of intracranial pressure (ICP) monitoring and to establish which ICP-targetted therapies are being used in children with severe traumatic brain injury (TBI) in the United Kingdom. To evaluate current practice against recently published guidelines.

Design And Setting: Prospective data collection of clinical and demographic information from paediatric and adult intensive care units in the UK and Ireland admitting children (< 16 years) with TBI between February 2001 and August 2003.

Sympathetic paraganglioma presenting with Horner's syndrome in a child.

Paragangliomas of the head and neck arise from collections of cells from the neural crest associated with cranial or sympathetic nerves. Such lesions are rare in children. The majority of paragangliomas in the paediatric age groups have been familial, occurring in association with an adrenal pheochromocytoma.

Health-related quality of life in adolescents with juvenile idiopathic arthritis.

Objective: To describe the health-related quality of life (HRQOL) of adolescents with juvenile idiopathic arthritis (JIA), and to examine the usefulness of the Juvenile Arthritis Quality of Life Questionnaire (JAQQ) in a UK context. It was hypothesized that HRQOL would decrease with worsening disease and disability.

Methods: Patients with JIA ages 11, 14, and 17 years were recruited from 10 major rheumatology centers.

Growing up and moving on in rheumatology: parents as proxies of adolescents with juvenile idiopathic arthritis.

Objective: To examine agreement about physical health, functional ability, and health-related quality of life (HRQOL) between adolescents with juvenile idiopathic arthritis (JIA) and their parents.

Methods: The study group comprised 303 adolescent-parent dyads who completed individual questionnaires, including the Childhood Health Assessment Questionnaire with visual analog scales for pain and general well-being, and the Juvenile Arthritis Quality of Life Questionnaire. Agreement was determined using the Bland and Altman method.

Predicting the performance of mitral prostheses implanted in children under 5 years of age.

Background: Mitral valve replacement (MVR) is occasionally indicated in infants and young children, necessitating the use of small prostheses. The performance of these small valves during somatic growth of the patient can lead to patient-prosthesis mismatch. This study examines performance of these valves over time to establish predicted performance and timing of replacement.

Growing up and moving on in rheumatology: development and preliminary evaluation of a transitional care programme for a multicentre cohort of adolescents with juvenile idiopathic arthritis.

This article describes the development and initial evaluation of an evidence-based transitional care programme recently implemented in a multicentre controlled trial in the United Kingdom. The individual components of the programme are described. Evaluation of the acceptability and utilization of these components employed questionnaires administered to users (adolescents with juvenile idiopathic arthritis and their parents) and providers (rheumatology health professionals).

Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience.

Objective: To describe a 12 year experience with staged surgical management of the hypoplastic left heart syndrome (HLHS) and to identify the factors that influenced outcome.

Methods: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure (median age 4 days, range 0-217 days). Subsequently 203 patients underwent a bidirectional Glenn procedure (stage II) and 81 patients underwent a modified Fontan procedure (stage III).

The RV-PA conduit stimulates better growth of the pulmonary arteries in hypoplastic left heart syndrome.

Objective: This retrospective study compared the size of the central pulmonary arteries in patients with hypoplastic left heart syndrome (HLHS) following either a classical Norwood or Norwood procedure with a right ventricle to pulmonary artery (RV-PA) conduit.

Methods: Between May 2001 and May 2003, 30 patients with HLHS underwent cardiac catheterization prior to stage II palliation. Patients were initially palliated with a classical Norwood (Classical group, n=18) or Norwood procedure with RV-PA conduit (RV-PA group; n=12).