Epidemiology of biliary atresia in England and Wales (1999-2006).

OBJECTIVE: To identify the epidemiological characteristics of infants with biliary atresia in England and Wales, since centralisation of its management in 1999. METHODS: The care of infants with biliary atresia (BA) in England and Wales is centralised to only three centres. All infants (treated from January 1999 to December 2006) were identified from a prospective national database; demographic details were ascertained from medical records and compared between two groups based on presumed aetiology (isolated biliary atresia (IBA) and developmental biliary atresia (DBA) (for example, syndromic infants, biliary atresia splenic malformation, cystic biliary atresia)). RESULTS: There were 302 (133 male (44%)) infants with BA that could be divided into IBA (n = 219, 73%) and DBA (n = 76, 25%). The overall incidence was 0.58/10 000 (1 in 17,049) live births with marked regional differences along a north-west/south-east axis varying from 0.38 (north-west England) to 0.78 (south-east England)/10,000 live births (OR 2.05 (95% CI 1.26-3.41); p = 0.002). The commonest month of birth was September with December being the least common, although there was no evidence for significant seasonal variation (p = 0.2). Infants with DBA were more likely to be female (p<0.001), of white background (p = 0.01), first-born (p = 0.04) and to be formula-fed (p = 0.07). Infants of south Asian origin came to surgery at an older age (59 (IQ 45-75) versus 52 (IQ 42-65) days; p = 0.03). CONCLUSIONS: There is a remarkable variation of incidence of biliary atresia within England and Wales, some of which may have been caused by factors related to a different aetiological and racial background.

Elective delayed midgut reduction-No anesthesia for gastroschisis: Selection and conversion criteria.

BACKGROUND/PURPOSE: In 1998 Bianchi and Dickson published their proposal for elective delayed midgut reduction without anaesthesia (EDMR-No GA). The study has been prospectively extended to develop "selection and conversion criteria" to ensure safe application of the technique. METHODS: In a prospective study from 1993 to date, EDMR-No GA was considered as first-line postnatal management in 35 children. The protocol and technique were those described by Bianchi and Dickson in 1998. RESULTS: EDMR-No GA was completed successfully in 25 children, of whom, 23 are normal with an aesthetic, scarless abdomen. Two children had bowel necrosis and died of short bowel state. A trial EDMR-No GA was converted to a staged silo reduction in 2 children who are both alive and well. EDMR-No GA was considered contraindicated in 8. Five had an elective, staged silo reduction, 2 had EDMR under anesthesia, and one 27-week-gestation baby died of severe hypothermia and acidosis before any procedure. CONCLUSIONS: No single technique is applicable in all circumstances, and "selection and conversion criteria" are relevant to safe EDMR-No GA. These include poor general condition, significant vital organ anomaly, bowel-to-abdomen disproportion and "at risk" bowel circulation. The development of distress and progressive metabolic acidosis during and after EDMR-No GA, are indications for urgent conversion to avoid serious bowel injury. Experience with EDMR-No GA hones the surgeon's sensitivity in assessing abdominal.

The anterior mediastinal approach for management of tracheomalacia.

BACKGROUND: Tracheomalacia occurs as a primary developmental defect or may be secondary to vascular compression. It is common in association with esophageal atresia and tracheoesophageal fistula. Collapse of the weak trachea is a cause of recurrent respiratory symptoms but may be severe and life threatening. METHODS: Between 1978 and 1999 at Sheffield Children's Hospital and The Royal Manchester Children's Hospital, of 16 children with clinically significant symptoms of tracheomalacia 8 underwent combined aortopexy and tracheopexy, 1 had aortopexy alone, 4 only had a tracheopexy, and 3 had tracheal reinforcement with free costal cartilage ring grafts. The surgical approach was limited to a low cervical skin crease incision with a midline manubrial split providing extrapleural access to the anterior mediastinum and allowing for all surgery under direct unimpaired vision. RESULTS: Ten children did not require postoperative ventilatory support. Four underwent ventilation for a few hours or days. One child required CPAP for 4 months for residual tracheomalacia and a further child, who had 3 operations to insert 11 costal cartilage ring grafts, underwent ventilation intermittently for 6 months. Adequate tracheal patency could be verified by intraoperative tracheoscopy and was sustained postoperatively so that only 1 child with associated bilateral vocal cord paralysis came to tracheostomy. Four children required prolonged hospitalization because of residual tracheomalacia, 2 for bronchomalacia and 2 because of esophageal narrowing leading to further surgery. All other children were fit for discharge within 10 to 30 days of surgery. Long-term follow-up has confirmed sustained tracheal improvement and resolution of the life-threatening features of tracheomalacia. CONCLUSIONS: The authors recommend the low skin crease transmanubrial approach, as described by Vaishnav and MacKinnon, for tracheopexy, aortopexy and for tracheal reconstruction for tracheomalacia. The approach gives excellent access for surgery under direct vision through a relatively avascular plane. It is associated with less morbidity than a conventional thoracotomy and leaves a more acceptable aesthetic scar.

Feminizing genitoplasty for congenital adrenal hyperplasia: what happens at puberty?

PURPOSE: We document the postpubertal outcome of feminizing genitoplasty. MATERIALS AND METHODS: A total of 14 girls, mean age 13.1 years, with congenital adrenal hyperplasia were assessed under anesthesia by a pediatric urologist, plastic/reconstructive surgeon and gynecologist. Of these patients 13 had previously undergone feminizing genitoplasty in early childhood at 4 different specialist centers in the United Kingdom. RESULTS: The outcome of clitoral surgery was unsatisfactory (clitoral atrophy or prominent glans) in 6 girls, including 3 whose genitoplasty had been performed by 3 different specialist pediatric urologists. Additional vaginal surgery was necessary for normal comfortable intercourse in 13 patients. Fibrosis and scarring were most evident in those who had undergone aggressive attempts at vaginal reconstruction in infancy. CONCLUSIONS: These disappointing results, even in the hands of specialists, highlight the importance of late followup and challenge the prevailing assumption that total correction can be achieved with a single stage operation in infancy. Although simple exteriorization of a low vagina can reasonably be combined with cosmetic correction of virilized external genitalia in infancy, we now believe that in some cases it may be best to defer definitive reconstruction of the intermediate or high vagina until after puberty. The psychological issues surrounding sexuality in these patients are inadequately researched and poorly understood.

Aesthetics and lateral thoracotomy in the neonate.

BACKGROUND: Lateral thoracotomy is a relatively common procedure in the neonate. Early reports on the incidence of shoulder deformity, scoliosis, and winged scapula rapidly led to a muscle-sparing approach. Scar cosmesis, however, received less attention. Better education and increasing sophistication have led to a higher aesthetic expectation by the public. METHODS: Since 1988 the authors have combined both principles in the high axillary skin crease approach to lateral thoracotomy in 27 children with esophageal atresia and tracheoesophageal fistula and two children with a patent ductus arteriosus. RESULTS: Access was not restricted, and early healing was uncomplicated by wound breakdown or infection. Scar aesthetics is excellent and is enhanced by the natural tendency of the scar to migrate into the axilla. Long-term morbidity has been singularly absent. CONCLUSION: Parent appreciation and operator satisfaction have been noteworthy.

Rectal biopsy for Hirschsprung's disease: what is the optimum method?

During a 3-year period, 258 infants and children underwent rectal biopsy to exclude Hirschsprung's disease (HD) and related disorders; 32 (12%) were found to have HD. Major morbidity occurred in 3 (2%) of 148 patients undergoing rectal suction biopsy (RSB) and 22 (13%) of 168 suction biopsies were inadequate for diagnosis. In 102 children over 6 months of age, Storz rectal cup biopsy forceps were used with no significant morbidity and adequate biopsies were obtained in 96% of cases. Open rectal biopsy was performed in 8 patients. The RSB tube is safe and reliable, but attention to technique is important. For children over 6 months of age undergoing rectal biopsy for HD, the Storz rectal cup biopsy forceps yields superior results.

Duodenal web presenting with acute pancreatitis.

An 8-year-old boy presented with acute pancreatitis and was found to have a fenestrated duodenal web (windsock) and associated anomalies. After partial excision of the web and duodenoplasty, he has remained well with no further symptoms.