Conservative management of antenatally diagnosed cystic lung malformations.

AIM: To review the outcome of all antenatally diagnosed conservatively managed congenital lung malformations (CLMs) managed at our centre. METHODS: All patients diagnosed antenatally with cystic lung malformations from 2001 to 2011, at a tertiary referral paediatric surgical centre practising a policy of conservative management of asymptomatic cases, were retrospectively reviewed. Data were collected from medical case notes and radiology reports. Ethical approval was obtained from our institutional research and development department. RESULTS: The complete records of 74 fetuses antenatally diagnosed with CLM were reviewed. There were 72 live births, at a median gestation of 39.6 weeks. Emergency lobectomy was performed in one symptomatic neonate. Elective lobectomies were performed at parental request in three asymptomatic infants, one of whom had a family history of synovial sarcoma. Two patients developed pneumonia in the affected lobe during early childhood and proceeded to lobectomy at the age of 3 years. One patient with a bronchopulmonary sequestration required embolisation for cyanotic episodes. The remaining 65 patients have been conservatively managed to date, and none have required hospital admission. Less than a quarter report mild respiratory symptoms such as cough or wheeze. Median follow-up is 5 years. CONCLUSIONS: This retrospective cohort study of 74 consecutive CLMs diagnosed antenatally over a 10-year period demonstrates that most of these lesions will remain asymptomatic throughout childhood. Although the natural history of CLMs in later years remains to be elucidated, we hope that this report on medium-term outcomes will be useful to clinicians who undertake antenatal counselling and may inform the discussion on how best to manage these children.

Ephedrine hydrochloride: novel use in the management of resistant non-neurogenic daytime urinary incontinence in children.

OBJECTIVE: To determine whether the adrenoceptor agonist, ephedrine hydrochloride, is an effective treatment for resistant non-neurogenic daytime urinary incontinence in children. METHODS: From 2000 to 2010, eighteen children with resistant non-neurogenic daytime urinary incontinence were treated with oral ephedrine hydrochloride at our institution. Sixteen were female and two were male. Median age at treatment was 12 years (range 5-15 years). Two children had spina bifida occulta. There were no other co-morbidities. Multiple anticholinergics were prescribed and dose maximized to support a bladder and bowel training programme, without achieving continence in this resistant group of children. Pre-treatment urodynamics were normal in 10, but revealed an open bladder neck in 8 patients. None showed detrusor over-activity. Oral ephedrine hydrochloride was started at 7.5 mg or 15 mg twice daily and titrated up to a maximum of 30 mg four times daily according to response. RESULTS: Median follow-up was 7 years (range 6-8 years). Seventeen children (94%) reported improvement in symptoms and six (33%) achieved complete urinary continence. All patients maintained compliant bladders on post-treatment urodynamics. Seven of the 8 previously open bladder necks were closed. No patients reported any significant side effects. Patients with open bladder necks on pre-treatment urodynamics were more likely to show a full response to ephedrine (odds ratio 15; 95% CI 1.2-185.2). CONCLUSIONS: Oral ephedrine hydrochloride is an effective treatment for carefully selected children with resistant non-neurogenic daytime urinary incontinence.

Persistent non-visualisation of the fetal stomach: diagnostic and prognostic implications.

DESIGN: All fetuses diagnosed with 'absent stomach' at anomaly screening over an 8-year period were identified using the University College Hospital fetal medicine database. These were cross-referenced with records from the paediatric surgical unit at Great Ormond Street Hospital and pathology department at University College Hospital to ascertain postnatal or postmortem diagnosis and outcome in each case. RESULTS: Of the 84 cases identified, eight were found to have normal stomachs on subsequent antenatal scans, while 76 had persistent non-visualisation of the stomach. Underlying diagnoses included 24 gastro-intestinal tract and/or respiratory anomalies, 22 aneuploidies, six neuromuscular syndromes, three central nervous system anomalies, seven renal anomalies and five genetic syndromes. Seven cases had no identifiable postnatal abnormalities, 26 pregnancies were terminated and nine fetuses died in utero. Of the 33 live births, eight died in the neonatal period and three died in infancy. Only 28 survived into childhood. Two patients were lost to follow up. CONCLUSIONS: Persistent non-visualisation of the fetal stomach in the antenatal period was associated with a wide range of underlying diagnoses. In many cases, prognosis was poor. Only 37% of pregnancies resulted in liveborn infants surviving more than 6 months. The incidence of an abnormal karyotype was 29%. Diagnosis and outcome was normal in only 9.2% of cases. We propose an algorithm for the management of persistent non-visualisation of the fetal stomach on antenatal ultrasound.

Pediatric laparoscopic-assisted gastric transposition: early experience and outcomes.

astric transposition in children is a well-established treatment for long-gap esophageal atresia and long caustic strictures when preservation of the native esophagus is impossible, but does involve laparotomy and, often, thoracotomy incisions. The minimally invasive approach to this operation has been attempted in 7 patients to date in the UK, all at Great Ormond Street Hospital (London, UK). Four patients were male, 3 were female, with a mean age of 3 years and 7 months (range, 5 months to 13 years). Indications were long-gap esophageal atresia where primary anastomosis was impossible (5 patients) and caustic ingestion with long esophageal strictures (2 patients). The stomach was mobilized laparoscopically by using 5-mm instruments and a pneumoperitoneum of 10 mm Hg. Following pyloromyotomy or pyloroplasty, a tunnel in the posterior mediastinum was developed. The fundus of the stomach was drawn to the neck and sutured to the cervical esophagus. The patients were ventilated on our intensive care unit and then returned to the surgical ward before discharge. Gastric transposition was completed laparoscopically in 6 of 7 patients. The mean duration of surgery was 327 minutes (range, 240-455). All patients survived. Minor complications were pneumonia (2), pleural effusion (1), and hiatus hernia (2). The mean hospital stay was 16 days (range, 8-36). Our preliminary experience would suggest that laparoscopic gastric transposition is a safe alternative to open surgery with satisfactory results.